The hemidesmosome: a target in auto-immune bullous disease.
Hemidesmosomes are subcellular structures that are involved in the attachment of a variety of epithelial cells, including basal epidermal keratinocytes, to the extracellular matrix. Like desmosomes, they provide a link between the intracytoplasmic keratin intermediate filaments and the plasma membrane. Major hemidesmosome plaque components include the bullous pemphigoid antigens of 230 and 180 kD and the alpha 6 beta 4-integrin. These transmembrane proteins may associate extracellularly with anchoring filament components including the 19-DEJ-1 proteoglycan and the polymeric proteins nicein/kalinin and epiligrin. Several of these hemidesmosome-related components have been implicated as targets for circulating auto-antibodies in immuno bullous disorders.
Eady
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St. John's Institute of Dermatology, United Medical School, University of London, UK.
D000937:Antigen-Antibody Reactions; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000032:analysis; D001327:Autoimmune Diseases / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D002352:Carrier Proteins; D015815:Cell Adhesion Molecules / Q000032:analysis; D003094:Collagen; D003598:Cytoskeletal Proteins; D003896:Desmosomes / Q000737:chemistry / Q000276:immunology / Q000648:ultrastructure*; D000071821:Dystonin; D006801:Humans; D016023:Integrins / Q000032:analysis; D015603:Keratinocytes / Q000648:ultrastructure; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D012872:Skin Diseases, Vesiculobullous / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D000097794:Kalinin; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D015815:Cell Adhesion Molecules; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D016023:Integrins; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097794:Kalinin; D000097805:Collagen Type XVII; C486268:DST protein, human