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

doi:10.1016/s0092-8674(03)00273-3

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

Professor Colin Jahoda colin.jahoda@durham.ac.uk
Professor

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