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Desmoglein 4 in hair follicle differentiation and epidermal adhesion: Evidence from inherited hypotrichosis and acquired pemphigus vulgaris

Kljuic, A; Bazzi, H; Sundberg, JP; Martinez-Mir, A; O'Shaughnessy, R; Mahoney, MG; Levy, M; Montagutelli, X; Ahmad, W; Aita, VM; Gordon, D; Uitto, J; Whiting, D; Ott, J; Fischer, S; Gilliam, TC; Jahoda, CAB; Morris, RJ; Panteleyev, AA; Nguyen, VT; Christiano, AM

Authors

A Kljuic

H Bazzi

JP Sundberg

A Martinez-Mir

R O'Shaughnessy

MG Mahoney

M Levy

X Montagutelli

W Ahmad

VM Aita

D Gordon

J Uitto

D Whiting

J Ott

S Fischer

TC Gilliam

RJ Morris

AA Panteleyev

VT Nguyen

AM Christiano



Abstract

Cell adhesion and communication are interdependent aspects of cell behavior that are critical for morphogenesis and tissue architecture. In the skin, epidermal adhesion is mediated in part by specialized cell-cell junctions known as desmosomes, which are characterized by the presence of desmosomal cadherins, known as desmogleins and desmocollins. We identified a cadherin family member, desmoglein 4, which is expressed in the suprabasal epidermis and hair follicle. The essential role of desmoglein 4 in skin was established by identifying mutations in families with inherited hypotrichosis, as well as in the lanceolate hair mouse. We also show that DSG4 is an autoantigen in pemphigus vulgaris. Characterization of the phenotype of naturally occurring mutant mice revealed disruption of desmosomal adhesion and perturbations in keratinocyte behavior. We provide evidence that desmoglein 4 is a key mediator of keratinocyte cell adhesion in the hair follicle, where it coordinates the transition from proliferation to differentiation.

Citation

Kljuic, A., Bazzi, H., Sundberg, J., Martinez-Mir, A., O'Shaughnessy, R., Mahoney, M., …Christiano, A. (2003). Desmoglein 4 in hair follicle differentiation and epidermal adhesion: Evidence from inherited hypotrichosis and acquired pemphigus vulgaris. Cell, 113(2), 249-260. https://doi.org/10.1016/s0092-8674%2803%2900273-3

Journal Article Type Article
Publication Date Apr 1, 2003
Deposit Date May 16, 2007
Journal Cell
Print ISSN 0092-8674
Publisher Elsevier
Peer Reviewed Peer Reviewed
Volume 113
Issue 2
Pages 249-260
DOI https://doi.org/10.1016/s0092-8674%2803%2900273-3




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