" /> Collagen Alpha-1(XVII) Chain - CISMeF





Preferred Label : Collagen Alpha-1(XVII) Chain;

NCIt synonyms : 180 kDa Bullous Pemphigoid Antigen 2; Collagen Type XVII Alpha 1 Polypeptide; COL17A1; Type XVII Collagen Alpha-1; Alpha 1 Type XVII Collagen;

NCIt related terms : Collagen Type XVII Alpha 1 Chain; BASEMENT MEMBRANE ZONE BP180; BP180; BMZ BP180; BPAG2; Bullous Pemphigoid Antigen 2;

NCIt definition : Collagen alpha-1(XVII) chain (1497 aa, 150 kDa) is encoded by the human COL17A1 gene. This protein plays a role in the attachment of keratinocytes to the basement membrane.;

Alternative definition : CDISC: An antigen encoded by the COL17A1 gene and is involved in the attachment of keratinocytes to the basement membrane. It can be considered as an autoantigen and is commonly associated with the autoimmune disease bullous pemphigoid.;

NCIt note : Collagen alpha-1(XVII) chain is cleaved by membrane-bound metalloproteases in keratinocytes, this leads to the shedding of a 120 kDa soluble form, 120 kDa linear IgA disease antigen (120 kDa linear IgA dermatosis antigen; linear IgA disease antigen 1; LAD-1), while a 97 kDa form, 97 kDa linear IgA disease antigen (97 kDa linear IgA bullous dermatosis antigen; 97 kDa LAD antigen; linear IgA bullous disease antigen of 97 kDa; 97-LAD; LABD97), remains membrane bound. Both the 120 and 97 kDa forms are major antigenic targets of autoantibodies in patients with linear IgA disease (LAD). (UniProt);

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14/05/2024


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