1. Desmoglein 4 in hair follicle differentiation and epidermal adhesion: evidence from inherited hypotrichosis and acquired pemphigus vulgaris.
- Author
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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 CA, Morris RJ, Panteleyev AA, Nguyen VT, and Christiano AM
- Subjects
- Animals, Cadherins genetics, Cadherins immunology, Chromosome Mapping, Chromosomes, Human, Pair 18 genetics, Cytoskeletal Proteins genetics, Cytoskeletal Proteins immunology, DNA Mutational Analysis, Desmogleins, Epidermis metabolism, Epidermis pathology, Female, Hair Follicle metabolism, Hair Follicle pathology, Humans, Hypertrichosis genetics, Hypertrichosis immunology, Male, Mice, Mice, Knockout, Mice, Mutant Strains, Microscopy, Electron, Molecular Sequence Data, Mutation genetics, Pedigree, Pemphigus genetics, Pemphigus immunology, Phenotype, Cadherins metabolism, Cell Adhesion genetics, Cell Differentiation genetics, Cytoskeletal Proteins metabolism, Epidermis embryology, Hair Follicle embryology, Hypertrichosis metabolism, Pemphigus metabolism
- 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.
- Published
- 2003
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