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The major inducible small heat shock protein HSP20-3 in the tardigrade Ramazzottius varieornatus forms filament-like structures and is an active chaperone. (2023)
Journal Article
Al-Ansari, M., Fitzsimons, T., Wei, W., Goldberg, M. W., Kunieda, T., & Quinlan, R. A. (2024). The major inducible small heat shock protein HSP20-3 in the tardigrade Ramazzottius varieornatus forms filament-like structures and is an active chaperone. Cell Stress and Chaperones, 29(1), 51-65. https://doi.org/10.1016/j.cstres.2023.12.001

The tardigrade Ramazzottius varieornatus has remarkable resilience to a range of environmental stresses. In this study, we have characterised two members of the small heat shock protein (sHSP) family in R. varieornatus, HSP20-3 and HSP20-6. These are... Read More about The major inducible small heat shock protein HSP20-3 in the tardigrade Ramazzottius varieornatus forms filament-like structures and is an active chaperone..

The redox-responsive roles of intermediate filaments in cellular stress detection, integration and mitigation (2023)
Journal Article
Pérez-Sala, D., & Quinlan, R. A. (2024). The redox-responsive roles of intermediate filaments in cellular stress detection, integration and mitigation. Current Opinion in Cell Biology, 86, Article 102283. https://doi.org/10.1016/j.ceb.2023.102283

Intermediate filaments are critical for cell and tissue homeostasis and for stress responses. Cytoplasmic intermediate filaments form versatile and dynamic assemblies that interconnect cellular organelles, participate in signaling and protect cells a... Read More about The redox-responsive roles of intermediate filaments in cellular stress detection, integration and mitigation.

Evaluation of cataract formation in fish exposed to environmental radiation at Chernobyl and Fukushima (2023)
Journal Article
Lerebours, A., Regini, J., Quinlan, R. A., Wada, T., Pierscionek, B., Devonshire, M., …Smith, J. T. (2023). Evaluation of cataract formation in fish exposed to environmental radiation at Chernobyl and Fukushima. Science of the Total Environment, 902, Article 165957. https://doi.org/10.1016/j.scitotenv.2023.165957

Recent studies apparently finding deleterious effects of radiation exposure on cataract formation in birds and voles living near Chernobyl represent a major challenge to current radiation protection regulations. This study conducted an integrated ass... Read More about Evaluation of cataract formation in fish exposed to environmental radiation at Chernobyl and Fukushima.

Multimorbidity due to novel pathogenic variants in theWFS1/RP1/NOD2genes: autosomal dominant congenital lamellar cataract, retinitis pigmentosa and Crohn’s disease in a British family (2023)
Journal Article
Berry, V., Ionides, A., Georgiou, M., Quinlan, R. A., & Michaelides, M. (2023). Multimorbidity due to novel pathogenic variants in theWFS1/RP1/NOD2genes: autosomal dominant congenital lamellar cataract, retinitis pigmentosa and Crohn’s disease in a British family. BMJ Open Ophthalmology, 8(1), Article e001252. https://doi.org/10.1136/bmjophth-2023-001252

Background A five generation family has been analysed by whole exome sequencing (WES) for genetic associations with the multimorbidities of congenital cataract (CC), retinitis pigmentosa (RP) and Crohn’s disease (CD). Methods WES was performed for un... Read More about Multimorbidity due to novel pathogenic variants in theWFS1/RP1/NOD2genes: autosomal dominant congenital lamellar cataract, retinitis pigmentosa and Crohn’s disease in a British family.

Independent Membrane Binding Properties of the Caspase Generated Fragments of the Beaded Filament Structural Protein 1 (BFSP1) Involves an Amphipathic Helix (2023)
Journal Article
Jarrin, M., Kalligeraki, A. A., Uwineza, A., Cawood, C. S., Brown, A. P., Ward, E. N., …Quinlan, R. A. (2023). Independent Membrane Binding Properties of the Caspase Generated Fragments of the Beaded Filament Structural Protein 1 (BFSP1) Involves an Amphipathic Helix. Cells, https://doi.org/10.3390/cells12121580

Background: BFSP1 (beaded filament structural protein 1) is a plasma membrane, Aqua- 21 porin 0 (AQP0/MIP)-associated intermediate filament protein expressed in the eye lens. BFSP1 is 22 myristoylated, a post-translation modification that requires ca... Read More about Independent Membrane Binding Properties of the Caspase Generated Fragments of the Beaded Filament Structural Protein 1 (BFSP1) Involves an Amphipathic Helix.

Identification and quantification of ionising radiation-induced oxysterol formation in membranes of lens fibre cells (2022)
Journal Article
Uwineza, A., Cummins, I., Jarrin, M., Kalligeraki, A. K., Barnard, S., Mol, M., …Quinlan, R. A. (2023). Identification and quantification of ionising radiation-induced oxysterol formation in membranes of lens fibre cells. Advances in Redox Research, 7, Article 100057. https://doi.org/10.1016/j.arres.2022.100057

Ionising radiation (IR) is a cause of lipid peroxidation, and epidemiological data have revealed a correlation between exposure to IR and the development of eye lens cataracts. Cataracts remain the leading cause of blindness around the world. The pla... Read More about Identification and quantification of ionising radiation-induced oxysterol formation in membranes of lens fibre cells.

Insights into the biochemical and biophysical mechanisms mediating the longevity of the transparent optics of the eye lens (2022)
Journal Article
Quinlan, R. A., & Clark, J. I. (2022). Insights into the biochemical and biophysical mechanisms mediating the longevity of the transparent optics of the eye lens. Journal of Biological Chemistry, 298(11), Article 102537. https://doi.org/10.1016/j.jbc.2022.102537

In the human eye, a transparent cornea and lens combine to form the “refracton” to focus images on the retina. This requires the refracton to have a high refractive index “n,” mediated largely by extracellular collagen fibrils in the corneal stroma a... Read More about Insights into the biochemical and biophysical mechanisms mediating the longevity of the transparent optics of the eye lens.

A recurrent variant in LIM2 causes an isolated congenital sutural/lamellar cataract in a Japanese family (2022)
Journal Article
Berry, V., Fujinami, K., Mochizuki, K., Iwata, T., Pontikos, N., Quinlan, R. A., & Michaelides, M. (2022). A recurrent variant in LIM2 causes an isolated congenital sutural/lamellar cataract in a Japanese family. Ophthalmic Genetics, 43(5), 622-626. https://doi.org/10.1080/13816810.2022.2090010

Background Genetically determined cataract is both clinically and molecularly highly heterogeneous. Here, we have identified a heterozygous variant in the lens integral membrane protein LIM2, the second most abundant protein in the lens, responsible... Read More about A recurrent variant in LIM2 causes an isolated congenital sutural/lamellar cataract in a Japanese family.

Cluster analyses of the TCGA and a TMA dataset using the coexpression of HSP27 and CRYAB improves alignment with clinical-pathological parameters of breast cancer and suggests different epichaperome influences for each sHSP (2022)
Journal Article
Quinlan, P. R., Figeuredo, G., Mongan, N., Jordan, L. B., Bray, S. E., Sreseli, R., …Quinlan, R. A. (2022). Cluster analyses of the TCGA and a TMA dataset using the coexpression of HSP27 and CRYAB improves alignment with clinical-pathological parameters of breast cancer and suggests different epichaperome influences for each sHSP. Cell Stress and Chaperones, 27(2), 177-188. https://doi.org/10.1007/s12192-022-01258-0

Our cluster analysis of the Cancer Genome Atlas for co-expression of HSP27 and CRYAB in breast cancer patients identified three patient groups based on their expression level combination (high HSP27+low CRYAB; low HSP27+high CRYAB; similar HSP27+CRYA... Read More about Cluster analyses of the TCGA and a TMA dataset using the coexpression of HSP27 and CRYAB improves alignment with clinical-pathological parameters of breast cancer and suggests different epichaperome influences for each sHSP.

The importance of the epithelial fibre cell interface to lens regeneration in an in vivo rat model and in a human bag-in-the-lens (BiL) sample (2021)
Journal Article
Wu, W., Lois, N., Prescott, A. R., Brown, A. P., Van Gerwen, V., Tassignon, M., …Quinlan, R. A. (2021). The importance of the epithelial fibre cell interface to lens regeneration in an in vivo rat model and in a human bag-in-the-lens (BiL) sample. Experimental Eye Research, 213, Article 108808. https://doi.org/10.1016/j.exer.2021.108808

Human lens regeneration and the Bag-in-the-Lens (BIL) surgical treatment for cataract both depend upon lens capsule closure for their success. Our studies suggest that the first three days after surgery are critical to their long-term outcomes. Using... Read More about The importance of the epithelial fibre cell interface to lens regeneration in an in vivo rat model and in a human bag-in-the-lens (BiL) sample.

Pathogenic variants in the CYP21A2 gene cause isolated autosomal dominant congenital posterior polar cataracts (2021)
Journal Article
Berry, V., Pontikos, N., Ionides, A., Kalitzeos, A., Quinlan, R. A., & Michaelides, M. (2022). Pathogenic variants in the CYP21A2 gene cause isolated autosomal dominant congenital posterior polar cataracts. Ophthalmic Genetics, 43(2), 218-223. https://doi.org/10.1080/13816810.2021.1998556

Background: Congenital cataracts are the most common cause of visual impairment worldwide. Inherited cataract is a clinically and genetically heterogeneous disease. Here we report disease-causing variants in a novel gene, CYP21A2, causing autosomal d... Read More about Pathogenic variants in the CYP21A2 gene cause isolated autosomal dominant congenital posterior polar cataracts.

Variants in PAX6, PITX3 and HSF4 causing autosomal dominant congenital cataracts (2021)
Journal Article
Berry, V., Ionides, A., Pontikos, N., Moore, A. T., Quinlan, R. A., & Michaelides, M. (2022). Variants in PAX6, PITX3 and HSF4 causing autosomal dominant congenital cataracts. Eye, 36(8), 1694-1701. https://doi.org/10.1038/s41433-021-01711-x

Background: Lens development is orchestrated by transcription factors. Disease-causing variants in transcription factors and their developmental target genes are associated with congenital cataracts and other eye anomalies. Methods: Using whole exome... Read More about Variants in PAX6, PITX3 and HSF4 causing autosomal dominant congenital cataracts.

Lens Epithelial Cell Proliferation in Response to Ionizing Radiation (2021)
Journal Article
Barnard, S., Uwineza, A., Kalligeraki, A., McCarron, R., Kruse, F., Ainsbury, E., & Quinlan, R. (2021). Lens Epithelial Cell Proliferation in Response to Ionizing Radiation. Radiation research, 197(1), 92-99. https://doi.org/10.1667/rade-20-00294.1

Lens epithelial cell proliferation and differentiation are naturally well regulated and controlled, a characteristic essential for lens structure, symmetry and function. The effect of ionizing radiation on lens epithelial cell proliferation has been... Read More about Lens Epithelial Cell Proliferation in Response to Ionizing Radiation.

On the Nature of Murine Radiation-Induced Subcapsular Cataracts: Optical Coherence Tomography-Based Fine Classification, In Vivo Dynamics and Impact on Visual Acuity (2021)
Journal Article
Pawliczek, D., Fuchs, H., Gailus-Durner, V., de Angelis, M. H., Quinlan, R., Graw, J., & Dalke, C. (2022). On the Nature of Murine Radiation-Induced Subcapsular Cataracts: Optical Coherence Tomography-Based Fine Classification, In Vivo Dynamics and Impact on Visual Acuity. Radiation research, 197(1), 7-21. https://doi.org/10.1667/rade-20-00163.1

Ionizing radiation is widely known to induce various kinds of lens cataracts, of which posterior subcapsular cataracts (PSCs) have the highest prevalence. Despite some studies regarding the epidemiology and biology of radiation-induced PSCs, the mech... Read More about On the Nature of Murine Radiation-Induced Subcapsular Cataracts: Optical Coherence Tomography-Based Fine Classification, In Vivo Dynamics and Impact on Visual Acuity.

Joining European Scientific Forces to Face Pandemics (2020)
Journal Article
Vasconcelos, M. H., Alcaro, S., Arechavala-Gomeza, V., Baumbach, J., Borges, F., Brevini, T. A., …Riganti, C. (2021). Joining European Scientific Forces to Face Pandemics. Trends in Microbiology, 29(2), 92-97. https://doi.org/10.1016/j.tim.2020.10.008

Despite the international guidelines on the containment of the coronavirus disease 2019 (COVID-19) pandemic, the European scientific community was not sufficiently prepared to coordinate scientific efforts. To improve preparedness for future pandemic... Read More about Joining European Scientific Forces to Face Pandemics.

The genetic landscape of crystallins in congenital cataract (2020)
Journal Article
Berry, V., Ionides, A., Pontikos, N., Georgiou, M., Yu, J., Ocaka, L. A., …Michaelides, M. (2020). The genetic landscape of crystallins in congenital cataract. Orphanet Journal of Rare Diseases, 15, Article 333. https://doi.org/10.1186/s13023-020-01613-3

Background: The crystalline lens is mainly composed of a large family of soluble proteins called the crystallins, which are responsible for its development, growth, transparency and refractive index. Disease-causing sequence variants in the crystalli... Read More about The genetic landscape of crystallins in congenital cataract.

Three-dimensional data capture and analysis of intact eye lenses evidences emmetropia-associated changes and strain-dependent differences in epithelial cell organization (2020)
Journal Article
Kalligeraki, A. A., Isted, A., Pal, R., Saunter, C., Girkin, J., Jarrin, M., …Quinlan, R. A. (2020). Three-dimensional data capture and analysis of intact eye lenses evidences emmetropia-associated changes and strain-dependent differences in epithelial cell organization. Scientific Reports, 10, Article 16898. https://doi.org/10.1038/s41598-020-73625-9

Organ and tissue development are highly coordinated processes; lens growth and functional integration into the eye (emmetropia) is a robust example. An epithelial monolayer covers the anterior hemisphere of the lens, and its organization is the key t... Read More about Three-dimensional data capture and analysis of intact eye lenses evidences emmetropia-associated changes and strain-dependent differences in epithelial cell organization.

Whole Exome Sequencing Reveals Novel and Recurrent Disease-Causing Variants in Lens Specific Gap Junctional Protein Encoding Genes Causing Congenital Cataract (2020)
Journal Article
Berry, V., Ionides, A., Pontikos, N., Moghul, I., Moore, A. T., Quinlan, R. A., & Michaelides, M. (2020). Whole Exome Sequencing Reveals Novel and Recurrent Disease-Causing Variants in Lens Specific Gap Junctional Protein Encoding Genes Causing Congenital Cataract. Genes, 11(5), Article 512. https://doi.org/10.3390/genes11050512

Pediatric cataract is clinically and genetically heterogeneous, and is the most common cause of childhood blindness worldwide. In this study, we aimed to identify disease-causing variants in three large British families and one isolated case with aut... Read More about Whole Exome Sequencing Reveals Novel and Recurrent Disease-Causing Variants in Lens Specific Gap Junctional Protein Encoding Genes Causing Congenital Cataract.

A novel missense mutation in LIM2 causing isolated autosomal dominant congenital cataract (2020)
Journal Article
Berry, V., Pontikos, N., Dudakova, L., Moore, A. T., Quinlan, R., Liskova, P., & Michaelides, M. (2020). A novel missense mutation in LIM2 causing isolated autosomal dominant congenital cataract. Ophthalmic Genetics, 41(2), 131-134. https://doi.org/10.1080/13816810.2020.1737950

Background: Congenital cataract is the most common cause of blindness in the world. Congenital cataracts are clinically and genetically heterogeneous and are mostly inherited in an autosomal dominant fashion. We identified the genetic cause of isolat... Read More about A novel missense mutation in LIM2 causing isolated autosomal dominant congenital cataract.

Site-specific phosphorylation and caspase cleavage of GFAP are new markers of Alexander Disease severity (2019)
Journal Article
Battaglia, R. A., Beltran, A. S., Delic, S., Dumitru, R., Robinson, J. A., Kabiraj, P., …Snider, N. T. (2019). Site-specific phosphorylation and caspase cleavage of GFAP are new markers of Alexander Disease severity. eLife, 8, Article e47789. https://doi.org/10.7554/elife.47789

Alexander Disease (AxD) is a fatal neurodegenerative disorder caused by mutations in glial fibrillary acidic protein (GFAP), which supports the structural integrity of astrocytes. Over 70 GFAP missense mutations cause AxD, but the mechanism linking d... Read More about Site-specific phosphorylation and caspase cleavage of GFAP are new markers of Alexander Disease severity.

Inverse dose-rate effect of ionising radiation on residual 53BP1 foci in the eye lens (2019)
Journal Article
Barnard, S., McCarron, R., Moquet, J., Quinlan, R., & Ainsbury, E. (2019). Inverse dose-rate effect of ionising radiation on residual 53BP1 foci in the eye lens. Scientific Reports, 9, Article 10418. https://doi.org/10.1038/s41598-019-46893-3

The influence of dose rate on radiation cataractogenesis has yet to be extensively studied. One recent epidemiological investigation suggested that protracted radiation exposure increases radiation-induced cataract risk: cumulative doses of radiation... Read More about Inverse dose-rate effect of ionising radiation on residual 53BP1 foci in the eye lens.

BFSP1 C-terminal domains released by post-translational processing events can alter significantly the calcium regulation of AQP0 water permeability (2019)
Journal Article
Tapodi, A., Clemens, D., Uwineza, A., Goldberg, M., Thinon, E., Heal, W., …Quinlan, R. (2019). BFSP1 C-terminal domains released by post-translational processing events can alter significantly the calcium regulation of AQP0 water permeability. Experimental Eye Research, 185, Article 107585. https://doi.org/10.1016/j.exer.2019.02.001

BFSP1 (beaded filament structural protein 1, filensin) is a cytoskeletal protein expressed in the eye lens. It binds AQP0 in vitro and its C-terminal sequences have been suggested to regulate the water channel activity of AQP0. A myristoylated fragme... Read More about BFSP1 C-terminal domains released by post-translational processing events can alter significantly the calcium regulation of AQP0 water permeability.

Cataractogenic load – a concept to study the contribution of ionizing radiation to accelerated aging in the eye lens (2019)
Journal Article
Uwineza, A., Kalligeraki, A. A., Hamada, N., Jarrin, M., & Quinlan, R. A. (2019). Cataractogenic load – a concept to study the contribution of ionizing radiation to accelerated aging in the eye lens. Mutation Research/Reviews in Mutation Research, 779, 68-81. https://doi.org/10.1016/j.mrrev.2019.02.004

Ionizing radiation (IR) damages DNA and other macromolecules, including proteins and lipids. Most cell types can repair DNA damage and cycle continuously their macromolecules as a mechanism to remove defective proteins and lipids. In those cells that... Read More about Cataractogenic load – a concept to study the contribution of ionizing radiation to accelerated aging in the eye lens.

Heat shock proteins are differentially expressed in brain and spinal cord: implications for multiple sclerosis (2018)
Journal Article
Gorter, R. P., Nutma, E., Jahreiβ, M., de Jonge, J. C., Quinlan, R., van der Valk, P., …Amor, S. (2018). Heat shock proteins are differentially expressed in brain and spinal cord: implications for multiple sclerosis. Clinical and Experimental Immunology, 194(2), 137-152. https://doi.org/10.1111/cei.13186

Aims: Multiple sclerosis (MS) is a chronic neurodegenerative disease characterised by demyelination, inflammation and neurodegeneration throughout the central nervous system. Although spinal cord pathology is an important factor contributing to disea... Read More about Heat shock proteins are differentially expressed in brain and spinal cord: implications for multiple sclerosis.

Non-invasive in vivo quantification of the developing optical properties and graded index of the embryonic eye lens using SPIM (2018)
Journal Article
Young, L., Jarrin, M., Saunter, C., Quinlan, R., & Girkin, J. (2018). Non-invasive in vivo quantification of the developing optical properties and graded index of the embryonic eye lens using SPIM. Biomedical Optics Express, 9(5), 2176-2188. https://doi.org/10.1364/boe.9.002176

Graded refractive index lenses are inherent to advanced visual systems in animals. By understanding their formation and local optical properties, significant potential for improved ocular healthcare may be realized. We report a novel technique measur... Read More about Non-invasive in vivo quantification of the developing optical properties and graded index of the embryonic eye lens using SPIM.

A rim-and-spoke hypothesis to explain the biomechanical roles for cytoplasmic intermediate filament networks (2017)
Journal Article
Quinlan, R., Schwartz, N., Windoffer, R., Richardson, C., Hawkins, T., Broussard, J., …Leube, R. (2017). A rim-and-spoke hypothesis to explain the biomechanical roles for cytoplasmic intermediate filament networks. Journal of Cell Science, 130(20), 3437-3445. https://doi.org/10.1242/jcs.202168

Textbook images of keratin intermediate filament (IF) networks in epithelial cells and the functional compromization of the epidermis by keratin mutations promulgate a mechanical role for this important cytoskeletal component. In stratified epithelia... Read More about A rim-and-spoke hypothesis to explain the biomechanical roles for cytoplasmic intermediate filament networks.

αB-crystallin is a sensor for assembly intermediates and for the subunit topology of desmin intermediate filaments (2017)
Journal Article
Sharma, S., Conover, G., Elliott, J., Perng, M., Herrmann, H., & Quinlan, R. (2017). αB-crystallin is a sensor for assembly intermediates and for the subunit topology of desmin intermediate filaments. Cell Stress and Chaperones, 22(4), 613-626. https://doi.org/10.1007/s12192-017-0788-7

Mutations in the small heat shock protein chaperone CRYAB (αB-crystallin/HSPB5) and the intermediate filament protein desmin, phenocopy each other causing cardiomyopathies. Whilst the binding sites for desmin on CRYAB have been determined, desmin epi... Read More about αB-crystallin is a sensor for assembly intermediates and for the subunit topology of desmin intermediate filaments.

The functional roles of the unstructured N- and C-terminal regions in alphaB-crystallin and other mammalian small heat-shock proteins (2017)
Journal Article
Carver, J., Grosas, A., Ecroyd, H., & Quinlan, R. (2017). The functional roles of the unstructured N- and C-terminal regions in alphaB-crystallin and other mammalian small heat-shock proteins. Cell Stress and Chaperones, 22(4), 627-638. https://doi.org/10.1007/s12192-017-0789-6

Small heat-shock proteins (sHsps), such as αB-crystallin, are one of the major classes of molecular chaperone proteins. In vivo, under conditions of cellular stress, sHsps are the principal defence proteins that prevent large-scale protein aggregatio... Read More about The functional roles of the unstructured N- and C-terminal regions in alphaB-crystallin and other mammalian small heat-shock proteins.

The impact of circadian rhythms on medical imaging and radiotherapy regimes for the paediatric patient (2016)
Journal Article
Forssell-Aronsson, E., & Quinlan, R. (2017). The impact of circadian rhythms on medical imaging and radiotherapy regimes for the paediatric patient. Radiation Protection Dosimetry, 173(1-3), 16-20. https://doi.org/10.1093/rpd/ncw328

Daily rhythmic changes are found in cellular events in cell cycle, DNA repair, apoptosis and angiogenesis in both normal and tumour tissue, as well as in enzymatic activity and drug metabolism. In this paper, we hypothesize that circadian rhythms nee... Read More about The impact of circadian rhythms on medical imaging and radiotherapy regimes for the paediatric patient.

Biophysical dissection of schistosome septins: Insights into oligomerization and membrane binding (2016)
Journal Article
Zeraik, A., Staykova, M., Fontes, M., Nemuraitė, I., Quinlan, R., Araújo, A., & DeMarco, R. (2016). Biophysical dissection of schistosome septins: Insights into oligomerization and membrane binding. Biochimie, 131, 96-105. https://doi.org/10.1016/j.biochi.2016.09.014

Septins are GTP-binding proteins that are highly conserved among eukaryotes and which are usually membrane-associated. They have been linked to several critical cellular functions such as exocytosis and ciliogenesis, but little mechanistic detail is... Read More about Biophysical dissection of schistosome septins: Insights into oligomerization and membrane binding.

The Lipidation Profile of Aquaporin-0 Correlates with The Acyl Composition of Phosphoethanolamine Lipids in Lens Membranes (2016)
Journal Article
Ismail, V. S., Mosely, J. A., Tapodi, A., Quinlan, R. A., & Sanderson, J. M. (2016). The Lipidation Profile of Aquaporin-0 Correlates with The Acyl Composition of Phosphoethanolamine Lipids in Lens Membranes. BBA - Biomembranes, 1858(11), 2763-2768. https://doi.org/10.1016/j.bbamem.2016.06.026

The lens fiber major intrinsic protein (otherwise known as aquaporin-0 (AQP0), MIP26 and MP26) has been examined by mass spectrometry (MS) in order to determine the speciation of acyl modifications to the side chains of lysine residues and the N-term... Read More about The Lipidation Profile of Aquaporin-0 Correlates with The Acyl Composition of Phosphoethanolamine Lipids in Lens Membranes.

Sub-nanometre mapping of the aquaporin-water interface with multifrequency atomic force microscopy (2016)
Journal Article
Ricci, M., Quinlan, R., & Voïtchovsky, K. (2017). Sub-nanometre mapping of the aquaporin-water interface with multifrequency atomic force microscopy. Soft Matter, 13(1), 187-195. https://doi.org/10.1039/c6sm00751a

Aquaporins are integral membrane proteins that regulate the transport of water and small molecules in and out of the cell. In eye lens tissue, circulation of water, ions and metabolites is ensured by a microcirculation system in which aquaporin-0 (AQ... Read More about Sub-nanometre mapping of the aquaporin-water interface with multifrequency atomic force microscopy.

Small molecules, both dietary and endogenous, influence the onset of lens cataracts (2016)
Journal Article
Barnes, S., & Quinlan, R. (2016). Small molecules, both dietary and endogenous, influence the onset of lens cataracts. Experimental Eye Research, 156, 87-94. https://doi.org/10.1016/j.exer.2016.03.024

How the lens ages successfully is a lesson in biological adaption and the emergent properties of its complement of cells and proteins. This living tissue contains some of the oldest proteins in our bodies and yet they remain functional for decades, d... Read More about Small molecules, both dietary and endogenous, influence the onset of lens cataracts.

Radiation protection of the eye lens in medical workers—basis and impact of the ICRP recommendations (2016)
Journal Article
Barnard, S., Ainsbury, E., Quinlan, R., & Buffler, S. (2016). Radiation protection of the eye lens in medical workers—basis and impact of the ICRP recommendations. British Journal of Radiology, 89(1060), Article 20151034. https://doi.org/10.1259/bjr.20151034

The aim of this article was to explore the evidence for the revised European Union basic safety standard (BSS) radiation dose limits to the lens of the eye, in the context of medical occupational radiation exposures. Publications in the open literatu... Read More about Radiation protection of the eye lens in medical workers—basis and impact of the ICRP recommendations.

In vivo, Ex Vivo, and In VitroApproaches to Study IntermediateFilaments in the Eye Lens (2015)
Journal Article
Jarrin, M., Young, L., Wu, W., Girkin, J., & Quinlan, R. (2016). In vivo, Ex Vivo, and In VitroApproaches to Study IntermediateFilaments in the Eye Lens. Methods in enzymology, 568, 581-611. https://doi.org/10.1016/bs.mie.2015.09.010

The role of the eye lens is to focus light into the retina. To perform this unique function, the ocular lens must be transparent. Previous studies have demonstrated the expression of vimentin, BFSP1, and BFSP2 in the eye lens. These intermediate fila... Read More about In vivo, Ex Vivo, and In VitroApproaches to Study IntermediateFilaments in the Eye Lens.

A dimensionless ordered pull-through model of the mammalian lens epithelium evidences scaling across species and explains the age-dependent changes in cell density in the human lens (2015)
Journal Article
Wu, J., Wu, W., Tholozan, F., Saunter, C., Girkin, J., & Quinlan, R. (2015). A dimensionless ordered pull-through model of the mammalian lens epithelium evidences scaling across species and explains the age-dependent changes in cell density in the human lens. Journal of the Royal Society. Interface, 12(108), https://doi.org/10.1098/rsif.2015.0391

We present a mathematical (ordered pull-through; OPT) model of the cell-density profile for the mammalian lens epithelium together with new experimental data. The model is based upon dimensionless parameters, an important criterion for inter-species... Read More about A dimensionless ordered pull-through model of the mammalian lens epithelium evidences scaling across species and explains the age-dependent changes in cell density in the human lens.

The lens of the eye: exposures in the UK medical sector and mechanistic studies of radiation effects (2015)
Journal Article
Bouffler, S., Peters, S., Gilvin, P., Slack, K., Markiewicz, E., Quinlan, R., …Ainsbury, E. (2015). The lens of the eye: exposures in the UK medical sector and mechanistic studies of radiation effects. Annals of the ICRP, 44(1 Suppl), 84-90. https://doi.org/10.1177/0146645314560693

The recommendation from the International Commission on Radiological Protection that the occupational equivalent dose limit for the lens of the eye should be reduced to 20 mSv year−1, averaged over 5 years with no year exceeding 50 mSv, has stimulate... Read More about The lens of the eye: exposures in the UK medical sector and mechanistic studies of radiation effects.

Nonlinear ionizing radiation-induced changes in eye lens cell proliferation, cyclin D1 expression and lens shape (2015)
Journal Article
Markiewicz, E., Barnard, S., Haines, J., Coster, M., Geel, O. V., Wu, W., …Quinlan, R. A. (2015). Nonlinear ionizing radiation-induced changes in eye lens cell proliferation, cyclin D1 expression and lens shape. Open Biology, 5, Article 150011. https://doi.org/10.1098/rsob.150011

Elevated cataract risk after radiation exposure was established soon after the discovery of X-rays in 1895. Today, increased cataract incidence among medical imaging practitioners and after nuclear incidents has highlighted how little is still unders... Read More about Nonlinear ionizing radiation-induced changes in eye lens cell proliferation, cyclin D1 expression and lens shape.

A silk purse from a sow’s ear – bioinspired materials based on α-helical coiled coils (2015)
Journal Article
Quinlan, R., Bromley, E., & Pohl, E. (2015). A silk purse from a sow’s ear – bioinspired materials based on α-helical coiled coils. Current Opinion in Cell Biology, 32, 131-137. https://doi.org/10.1016/j.ceb.2014.12.010

This past few years have heralded remarkable times for intermediate filaments with new revelations of their structural properties that has included the first crystallographic-based model of vimentin to build on the experimental data of intra-filament... Read More about A silk purse from a sow’s ear – bioinspired materials based on α-helical coiled coils.

A gradient of matrix-bound FGF-2 and perlecan is available to lens epithelial cells (2014)
Journal Article
Wu, W., Tholozan, F., Goldberg, M., Bowen, L., Wu, J., & Quinlan, R. (2014). A gradient of matrix-bound FGF-2 and perlecan is available to lens epithelial cells. Experimental Eye Research, 120, 10-14. https://doi.org/10.1016/j.exer.2013.12.004

Fibroblast growth factors play a key role in regulating lens epithelial cell proliferation and differentiation via an anteroposterior gradient that exists between the aqueous and vitreous humours. FGF-2 is the most important for lens epithelial cell... Read More about A gradient of matrix-bound FGF-2 and perlecan is available to lens epithelial cells.

Caspase cleavage of GFAP produces an assembly-compromised proteolytic fragment that promotes filament aggregation (2013)
Journal Article
Chen, M., Hagemann, T., Quinlan, R., Messing, A., & Perng, M. (2013). Caspase cleavage of GFAP produces an assembly-compromised proteolytic fragment that promotes filament aggregation. Conversations about pedagogy and teaching underpinned by research enquiry, 5(5), https://doi.org/10.1042/an20130032

IF (intermediate filament) proteins can be cleaved by caspases to generate proapoptotic fragments as shown for desmin. These fragments can also cause filament aggregation. The hypothesis is that disease-causing mutations in IF proteins and their subs... Read More about Caspase cleavage of GFAP produces an assembly-compromised proteolytic fragment that promotes filament aggregation.

Chaperones: needed for both the good times and the bad times (2013)
Journal Article
Quinlan, R., & Ellis, R. (2013). Chaperones: needed for both the good times and the bad times. Philosophical Transactions of the Royal Society B: Biological Sciences, 368(1617), Article 20130091. https://doi.org/10.1098/rstb.2013.0091

In this issue, we explore the assembly roles of protein chaperones, mainly through the portal of their associated human diseases (e.g. cardiomyopathy, cataract, neurodegeneration, cancer and neuropathy). There is a diversity to chaperone function tha... Read More about Chaperones: needed for both the good times and the bad times.

The specificity of the interaction between αB-crystallin and desmin filaments and its impact on filament aggregation and cell viability (2013)
Journal Article
Elliott, J., Der Perng, M., Prescott, A., Jansen, K., Koenderink, G., & Quinlan, R. (2013). The specificity of the interaction between αB-crystallin and desmin filaments and its impact on filament aggregation and cell viability. Philosophical Transactions of the Royal Society B: Biological Sciences, 368(1617), Article 20120375. https://doi.org/10.1098/rstb.2012.0375

CRYAB (αB-crystallin) is expressed in many tissues and yet the R120G mutation in CRYAB causes tissue-specific pathologies, namely cardiomyopathy and cataract. Here, we present evidence to demonstrate that there is a specific functional interaction of... Read More about The specificity of the interaction between αB-crystallin and desmin filaments and its impact on filament aggregation and cell viability.

Changes in the quaternary structure and function of MjHSP16.5 attributable to deletion of the I–X–I motif and introduction of the substitution, R107G in the a-crystallin domain (2013)
Journal Article
Quinlan, R., Zhang, Y., Lansbury, A., Williamson, I., Pohl, E., & Sun, F. (2013). Changes in the quaternary structure and function of MjHSP16.5 attributable to deletion of the I–X–I motif and introduction of the substitution, R107G in the a-crystallin domain. Philosophical Transactions of the Royal Society B: Biological Sciences, 368(1617), Article 20120327. https://doi.org/10.1098/rstb.2012.0327

The archael small heat-shock protein (sHSP), MjHSP16.5, forms a 24-subunit oligomer with octahedral symmetry. Here, we demonstrate that the IXI motif present in the C-terminal domain is necessary for the oligomerization of MjHSP16.5. Removal increase... Read More about Changes in the quaternary structure and function of MjHSP16.5 attributable to deletion of the I–X–I motif and introduction of the substitution, R107G in the a-crystallin domain.

Alexander disease causing mutations in the C-terminal domain of GFAP are deleterious both to assembly and network formation with the potential to both activate caspase 3 and decrease cell viability (2011)
Journal Article
Chen, Y., Lim, S., Chen, M., Quinlan, R. A., & Perng, M. (2011). Alexander disease causing mutations in the C-terminal domain of GFAP are deleterious both to assembly and network formation with the potential to both activate caspase 3 and decrease cell viability. Experimental Cell Research, 317(16), 2252-2266. https://doi.org/10.1016/j.yexcr.2011.06.017

Evolution of the vertebrate beaded filament protein, Bfsp2; comparing the in vitro assembly properties of a “tailed” zebrafish Bfsp2 to its “tailless” human orthologue. (2011)
Journal Article
Qu, B., Landsbury, A., Schoenthaler, H. B., Dahm, R., Liu, Y., Clark, J. I., …Quinlan, R. A. (2012). Evolution of the vertebrate beaded filament protein, Bfsp2; comparing the in vitro assembly properties of a “tailed” zebrafish Bfsp2 to its “tailless” human orthologue. Experimental Eye Research, 94(1), 192-202. https://doi.org/10.1016/j.exer.2011.12.001

In bony fishes, Bfsp2 orthologues are predicted to possess a C-terminal tail domain, which is absent from avian, amphibian and mammalian Bfsp2 sequences. These sequences, are however, not conserved between fish species and therefore questions whether... Read More about Evolution of the vertebrate beaded filament protein, Bfsp2; comparing the in vitro assembly properties of a “tailed” zebrafish Bfsp2 to its “tailless” human orthologue..

Multiple Sites in alpha B-Crystallin Modulate Its Interactions with Desmin Filaments Assembled In Vitro (2011)
Journal Article
Houck, S. A., Landsbury, A., Clark, J. I., & Quinlan, R. A. (2011). Multiple Sites in alpha B-Crystallin Modulate Its Interactions with Desmin Filaments Assembled In Vitro. PLoS ONE, 6(11), Article e25859. https://doi.org/10.1371/journal.pone.0025859

The β3- and β8-strands and C-terminal residues 155–165 of αB-crystallin were identified by pin arrays as interaction sites for various client proteins including the intermediate filament protein desmin. Here we present data using 5 well-characterised... Read More about Multiple Sites in alpha B-Crystallin Modulate Its Interactions with Desmin Filaments Assembled In Vitro.

Homeostasis in the vertebrate lens: mechanisms of solute exchange (2011)
Journal Article
Dahm, R., van Marle, J., Quinlan, R. A., Prescott, A. R., & Vrensen, G. F. (2011). Homeostasis in the vertebrate lens: mechanisms of solute exchange. Philosophical Transactions of the Royal Society B: Biological Sciences, 366(1568), 1265-1277. https://doi.org/10.1098/rstb.2010.0299

The eye lens is avascular, deriving nutrients from the aqueous and vitreous humours. It is, however, unclear which mechanisms mediate the transfer of solutes between these humours and the lens' fibre cells (FCs). In this review, we integrate the publ... Read More about Homeostasis in the vertebrate lens: mechanisms of solute exchange.

Oligomers of mutant glial fibrillary acidic protein (GFAP) Inhibit the proteasome system in alexander disease astrocytes, and the small heat shock protein αB-crystallin reverses the inhibition (2010)
Journal Article
Tang, G., Perng, M., Wilk, S., Quinlan, R., & Goldman, J. (2010). Oligomers of mutant glial fibrillary acidic protein (GFAP) Inhibit the proteasome system in alexander disease astrocytes, and the small heat shock protein αB-crystallin reverses the inhibition. Journal of Biological Chemistry, 285(14), 10527-10537. https://doi.org/10.1074/jbc.m109.067975

The accumulation of the intermediate filament protein, glial fibrillary acidic protein (GFAP), in astrocytes of Alexander disease (AxD) impairs proteasome function in astrocytes. We have explored the molecular mechanism that underlies the proteasome... Read More about Oligomers of mutant glial fibrillary acidic protein (GFAP) Inhibit the proteasome system in alexander disease astrocytes, and the small heat shock protein αB-crystallin reverses the inhibition.

alpha B-crystallin, vimentin and increased p53 expression levels in breast cancer is associated with poor prognosis (2009)
Journal Article
Quinlan, P., Sreseli, R., Quinlan, R., Hadad, S., Bray, S., Kernohan, N., …Thompson, A. (2009). alpha B-crystallin, vimentin and increased p53 expression levels in breast cancer is associated with poor prognosis. Cancer Research, 69(2 Suppl), 321S-322S. https://doi.org/10.1158/0008-5472.sabcs-5070

ackground: Previous studies have identified αB-crystallin as a marker of poor prognosis for breast cancer and have suggested that it is an excellent marker for tumours of basal origin. Increased expression of αB-crystallin has been associated with an... Read More about alpha B-crystallin, vimentin and increased p53 expression levels in breast cancer is associated with poor prognosis.

A cell polarity protein aPKCλ is required for eye lens formation and growth (2009)
Journal Article
Sugiyama, Y., Akimoto, K., Robinson, M. L., Ohno, S., & Quinlan, R. A. (2009). A cell polarity protein aPKCλ is required for eye lens formation and growth. Developmental Biology, 336(2), 246-256. https://doi.org/10.1016/j.ydbio.2009.10.010

The organisation of individual cells into a functional three-dimensional tissue is still a major question in developmental biology. Modulation of epithelial cell shape is a critical driving force in forming tissues. This is well illustrated in the ey... Read More about A cell polarity protein aPKCλ is required for eye lens formation and growth.

MAPKAPK-2 modulates p38-MAPK localization and small heat shock protein phosphorylation but does not mediate the injury associated with p38-MAPK activation during myocardial ischemia (2009)
Journal Article
Gorog, D. A., Jabr, R. I., Tanno, M., Sarafraz, N., Clark, J. E., Fisher, S. G., …Heads, R. J. (2009). MAPKAPK-2 modulates p38-MAPK localization and small heat shock protein phosphorylation but does not mediate the injury associated with p38-MAPK activation during myocardial ischemia. Cell Stress and Chaperones, 14(5), 477-489. https://doi.org/10.1007/s12192-009-0101-5

Glial Fibrillary Acidic Protein Filaments Can Tolerate the Incorporation of Assembly-compromised GFAP-delta, but with Consequences for Filament Organization and alpha B-Crystallin Association (2008)
Journal Article
Perng, M., Wen, S., Gibbon, T., Middeldorp, J., Sluijs, J., Hol, E. M., & Quinlan, R. A. (2008). Glial Fibrillary Acidic Protein Filaments Can Tolerate the Incorporation of Assembly-compromised GFAP-delta, but with Consequences for Filament Organization and alpha B-Crystallin Association. Molecular Biology of the Cell, 19(10), 4521-4533. https://doi.org/10.1091/mbc.e08-03-0284

Truncation of αB-crystallin by the myopathy-causing Q151X mutation significantly destabilizes the protein leading to aggregate formation in transfected cells (2008)
Journal Article
Hayes, V. H., Devlin, G., & Quinlan, R. A. (2008). Truncation of αB-crystallin by the myopathy-causing Q151X mutation significantly destabilizes the protein leading to aggregate formation in transfected cells. Journal of Biological Chemistry, 283(16), 10500-10512. https://doi.org/10.1074/jbc.m706453200

Here we investigate the effects of a myopathy-causing mutation in αB-crystallin, Q151X, upon its structure and function. This mutation removes the C-terminal domain of αB-crystallin, which is expected to compromise both its oligomerization and chaper... Read More about Truncation of αB-crystallin by the myopathy-causing Q151X mutation significantly destabilizes the protein leading to aggregate formation in transfected cells.

Lens cells: More than meets the eye (2007)
Journal Article
Tholozan, F., & Quinlan, R. (2007). Lens cells: More than meets the eye. International Journal of Biochemistry and Cell Biology, 39(10), 1754-1759

Lens cells originate from the head ectoderm and differentiate into an avascular organ constituted from two contiguous cell subpopulations of very different morphology. Lens cells, together with corneal cells, are responsible for the transmission and... Read More about Lens cells: More than meets the eye.

Insights into the beaded filament of the eye lens (2007)
Journal Article
Perng, M., Zhang, Q., & Quinlan, R. (2007). Insights into the beaded filament of the eye lens. Experimental Cell Research, 313(10), 2180-8

Filensin (BFSP1) and CP49 (BFSP2) represent two members of the IF protein superfamily that are thus far exclusively expressed in the eye lens. Mutations in both proteins cause lens cataract and careful consideration of the detail of these cataract ph... Read More about Insights into the beaded filament of the eye lens.

GFAP and its role in Alexander disease (2007)
Journal Article
Quinlan, R., Brenner, M., Goldman, J., & Messing, A. (2007). GFAP and its role in Alexander disease. Experimental Cell Research, 313(10), 2077-87

Here we review how GFAP mutations cause Alexander disease. The current data suggest that a combination of events cause the disease. These include: (i) the accumulation of GFAP and the formation of characteristic aggregates, called Rosenthal fibers, (... Read More about GFAP and its role in Alexander disease.

Regulation of contractility by Hsp27 and Hic-5 in rat mesenteric small arteries. (2007)
Journal Article
Srinivasan, R., Forman, S., Quinlan, R., Ohanian, J., & Ohanian, V. (2008). Regulation of contractility by Hsp27 and Hic-5 in rat mesenteric small arteries. American Journal of Physiology - Heart and Circulatory Physiology, 294(2), H961-H969. https://doi.org/10.1152/ajpheart.00939.2007

The regulation of small artery contractility by vasoconstrictors is important for vascular function, and actin cytoskeleton remodeling is required for contraction. p38 MAPK and tyrosine kinases are implicated in actin polymerization and contraction t... Read More about Regulation of contractility by Hsp27 and Hic-5 in rat mesenteric small arteries..

Reorganization of centrosomal marker proteins coincides with epithelial cell differentiation in the vertebrate lens (2007)
Journal Article
Dahm, R., Procter, J., Ireland, M., Lo, W., Mogensen, M., Quinlan, R., & Prescott, A. (2007). Reorganization of centrosomal marker proteins coincides with epithelial cell differentiation in the vertebrate lens. Experimental Cell Research, 85(5), 696-713. https://doi.org/10.1016/j.exer.2007.07.022

The differentiation of epithelial cells in the vertebrate lens involves a series of changes that includes the degradation of all intracellular organelles and a dramatic elongation of the cells. The latter is accompanied by a substantial remodelling o... Read More about Reorganization of centrosomal marker proteins coincides with epithelial cell differentiation in the vertebrate lens.

FGF-2 Release from the Lens Capsule by MMP-2 Maintains Lens Epithelial Cell Viability (2007)
Journal Article
Tholozan, F., Gribbon, C., Li, Z., Goldberg, M., Prescott, A., McKie, N., & Quinlan, R. (2007). FGF-2 Release from the Lens Capsule by MMP-2 Maintains Lens Epithelial Cell Viability. Molecular Biology of the Cell, 18(11), 4222-4231. https://doi.org/10.1091/mbc.e06-05-0416

The lens is an avascular tissue, separated from the aqueous and vitreous humors by its own extracellular matrix, the lens capsule. Here we demonstrate that the lens capsule is a source of essential survival factors for lens epithelial cells. Primary... Read More about FGF-2 Release from the Lens Capsule by MMP-2 Maintains Lens Epithelial Cell Viability.

The Alexander disease-causing Glial Fibrillary Acidic Protein mutant, R416W, accumulates into Rosenthal fibers by a pathway that involves filament aggregation and the association of alphaB-crystallin and HSP27 (2006)
Journal Article
Perng, M., Su, M., Wen, S., Li, R., Gibbon, T., Prescott, A., …Quinlan, R. (2006). The Alexander disease-causing Glial Fibrillary Acidic Protein mutant, R416W, accumulates into Rosenthal fibers by a pathway that involves filament aggregation and the association of alphaB-crystallin and HSP27. American Journal of Human Genetics, 79(2), 197-213. https://doi.org/10.1086/504411

Here, we describe the early events in the disease pathogenesis of Alexander disease. This is a rare and usually fatal neurodegenerative disorder whose pathological hallmark is the abundance of protein aggregates in astrocytes. These aggregates, terme... Read More about The Alexander disease-causing Glial Fibrillary Acidic Protein mutant, R416W, accumulates into Rosenthal fibers by a pathway that involves filament aggregation and the association of alphaB-crystallin and HSP27.

Desmin aggregate formation by R120G alpha B-crystallin is caused by altered filament interactions and is dependent upon network status in cells (2004)
Journal Article
Perng, M., Wen, S., van den Ijssel, P., Prescott, A., & Quinlan, R. (2004). Desmin aggregate formation by R120G alpha B-crystallin is caused by altered filament interactions and is dependent upon network status in cells. Molecular Biology of the Cell, 15(5), 2335-2346. https://doi.org/10.1091/mbc.e03-12-0893

The R120G mutation in alphaB-crystallin causes desmin-related myopathy. There have been a number of mechanisms proposed to explain the disease process, from altered protein processing to loss of chaperone function. Here, we show that the mutation alt... Read More about Desmin aggregate formation by R120G alpha B-crystallin is caused by altered filament interactions and is dependent upon network status in cells.

Lamin A/C binding protein LAP2 alpha is required for nuclear anchorageof retinoblastoma protein (2002)
Journal Article
Markiewicz, E., Dechat, T., Foisner, R., Quinlan, R., & Hutchison, C. (2002). Lamin A/C binding protein LAP2 alpha is required for nuclear anchorageof retinoblastoma protein. Molecular Biology of the Cell, 13(12), 4401-4413. https://doi.org/10.1091/mbc.e02-07-0450

The phosphorylation-dependent anchorage of retinoblastoma protein Rb in the nucleus is essential for its function. We show that its pocket C domain is both necessary and sufficient for nuclear anchorage by transiently expressing green fluorescent pro... Read More about Lamin A/C binding protein LAP2 alpha is required for nuclear anchorageof retinoblastoma protein.

Differential effect of simvastatin on activation of Rac(1) vs.activation of the heat shock protein 27-mediated pathway upon oxidativestress, in human smooth muscle cells (2002)
Journal Article
Negre-Aminou, P., van Leeuwen, R., van Thiel, G., van den IJssel, P., de Jong, W., Quinlan, R., & Cohen, L. (2002). Differential effect of simvastatin on activation of Rac(1) vs.activation of the heat shock protein 27-mediated pathway upon oxidativestress, in human smooth muscle cells. Biochemical Pharmacology, 64(10), 1483-1491

Altered aggregation properties of mutant gamma-crystallins cause inherited cataract (2002)
Journal Article
Sandilands, A., Hutcheson, A., Long, H., Prescott, A., Vrensen, G., Löster, J., …Quinlan, R. (2002). Altered aggregation properties of mutant gamma-crystallins cause inherited cataract. The EMBO Journal, 21(22), 6005-6014. https://doi.org/10.1093/emboj/cdf609

Protein inclusions are associated with a diverse group of human diseases ranging from localized neurological disorders through to systemic non-neuropathic diseases. Here, we present evidence that the formation of intranuclear inclusions is a key even... Read More about Altered aggregation properties of mutant gamma-crystallins cause inherited cataract.

Association of the nuclear matrix component NuMA with the Cajal bodyand nuclear speckle compartments during transitions in transcriptionalactivity in lens cell differentiation (2002)
Journal Article
Gribbon, C., Dahm, R., Prescott, A., & Quinlan, R. (2002). Association of the nuclear matrix component NuMA with the Cajal bodyand nuclear speckle compartments during transitions in transcriptionalactivity in lens cell differentiation. European Journal of Cell Biology, 81(10), 557-566. https://doi.org/10.1078/0171-9335-00275

The transcriptional status of cells can be deduced from the staining pattern of various nuclear markers such as the Cajal body, nucleolus and nuclear speckles. In this study we have used these markers to correlate transcriptional status with cell dif... Read More about Association of the nuclear matrix component NuMA with the Cajal bodyand nuclear speckle compartments during transitions in transcriptionalactivity in lens cell differentiation.

Alpha-b crystallin gene (CRYAB) mutation causes dominant congenital posterior polar cataract in humans (2001)
Journal Article
Berry, V., Francis, P., Reddy, M., Collyer, D., Vithana, E., MacKay, I., …Quinlan, R. (2001). Alpha-b crystallin gene (CRYAB) mutation causes dominant congenital posterior polar cataract in humans. American Journal of Human Genetics, 69(5), 1141-1145. https://doi.org/10.1086/324158

Congenital cataracts are an important cause of bilateral visual impairment in infants. In a four-generation family of English descent, we mapped dominant congenital posterior polar cataract to chromosome 11q22-q22.3. The maximum LOD score, 3.92 at re... Read More about Alpha-b crystallin gene (CRYAB) mutation causes dominant congenital posterior polar cataract in humans.

The cardiomyopathy and lens cataract mutation in αB-crystallin alters its protein structure, chaperone activity, and interaction withintermediate filaments in vitro (1999)
Journal Article
Der Perng, M., Muchowski, P., van den IJssel, P., Wu, G., Hutcheson, A., Clark, J., & Quinlan, R. (1999). The cardiomyopathy and lens cataract mutation in αB-crystallin alters its protein structure, chaperone activity, and interaction withintermediate filaments in vitro. Journal of Biological Chemistry, 274(47), 33235-33243. https://doi.org/10.1074/jbc.274.47.33235

Desmin-related myopathy and cataract are both caused by the R120G mutation in αB-crystallin. Desmin-related myopathy is one of several diseases characterized by the coaggregation of intermediate filaments with αB-crystallin, and it identifies interme... Read More about The cardiomyopathy and lens cataract mutation in αB-crystallin alters its protein structure, chaperone activity, and interaction withintermediate filaments in vitro.

cDNA Cloning, Expression, and Assembly Characteristics of Mouse Keratin 16 (1998)
Journal Article
Porter, R., Hutcheson, A., Rugg, E., Quinlan, R., & Lane, E. (1998). cDNA Cloning, Expression, and Assembly Characteristics of Mouse Keratin 16. Journal of Biological Chemistry, 273(48), 32265-32272. https://doi.org/10.1074/jbc.273.48.32265

There has been speculation as to the existence of the mouse equivalent of human type I keratin 16 (K16). The function of this keratin is particularly intriguing because, in normal epidermis, it is usually confined to hair follicles and only becomes e... Read More about cDNA Cloning, Expression, and Assembly Characteristics of Mouse Keratin 16.

MOLECULAR-INTERACTIONS IN PARACRYSTALS OF A FRAGMENT CORRESPONDING TOTHE ALPHA-HELICAL COILED-COIL ROD PORTION OF GLIAL FIBRILLARY ACIDICPROTEIN - EVIDENCE FOR AN ANTIPARALLEL PACKING OF MOLECULES ANDPOLYMORPHISM RELATED TO INTERMEDIATE FILAMENT STRUCTURE (1989)
Journal Article
Stewart, M., QUINLAN, R., & Moir, R. (1989). MOLECULAR-INTERACTIONS IN PARACRYSTALS OF A FRAGMENT CORRESPONDING TOTHE ALPHA-HELICAL COILED-COIL ROD PORTION OF GLIAL FIBRILLARY ACIDICPROTEIN - EVIDENCE FOR AN ANTIPARALLEL PACKING OF MOLECULES ANDPOLYMORPHISM RELATED TO INTERMEDIATE FILAMENT STRUCTURE. Journal of Cell Biology, 109(1), 225-234

SOME ASPECTS OF TUBULIN STRUCTURE AND TISSUE-SPECIFICITY (1984)
Journal Article
Little, M., Luduena, R., QUINLAN, R., Ponstingl, H., Krauhs, E., & Raff, E. (1984). SOME ASPECTS OF TUBULIN STRUCTURE AND TISSUE-SPECIFICITY. Journal of submicroscopic cytology and pathology, 16(1), 11-13

MOLECULAR-INTERACTIONS IN INTERMEDIATE-SIZED FILAMENTS REVEALED BYCHEMICAL CROSS-LINKING - HETEROPOLYMERS OF VIMENTIN AND GLIAL FILAMENTPROTEIN IN CULTURED HUMAN GLIOMA-CELLS (1983)
Journal Article
QUINLAN, R., & Franke, W. (1983). MOLECULAR-INTERACTIONS IN INTERMEDIATE-SIZED FILAMENTS REVEALED BYCHEMICAL CROSS-LINKING - HETEROPOLYMERS OF VIMENTIN AND GLIAL FILAMENTPROTEIN IN CULTURED HUMAN GLIOMA-CELLS. European journal of biochemistry (Internet), 132(3), 477-484. https://doi.org/10.1111/j.1432-1033.1983.tb07386.x

Heteropolymer Filaments of Vimentin and Desmin in Vascular Smooth Muscle Tissue and Cultured Baby Hamster Kidney Cells Demonstrated by Chemical Crosslinking. (1982)
Journal Article
Quinlan, R., & Franke, W. (1982). Heteropolymer Filaments of Vimentin and Desmin in Vascular Smooth Muscle Tissue and Cultured Baby Hamster Kidney Cells Demonstrated by Chemical Crosslinking. Proceedings of the National Academy of Sciences, 79(11), 3452-3456. https://doi.org/10.1073/pnas.79.11.3452

Certain smooth muscle cells of blood vessel walls as well as cultured baby hamster kidney cells contain simultaneously two different intermediate-sized filament (IF) proteins, desmin and vimentin. We have examined the question of the occurrence of bo... Read More about Heteropolymer Filaments of Vimentin and Desmin in Vascular Smooth Muscle Tissue and Cultured Baby Hamster Kidney Cells Demonstrated by Chemical Crosslinking..