Post Translational Modifications | The intracellular/endo domain is disulfide-linked. Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains. The ectodomain is shedded from the surface of keratinocytes resulting in a 120-kDa soluble form, also named as 120 kDa linear IgA disease antigen. The shedding is mediated by membrane-bound metalloproteases. This cleavage is inhibited by phosphorylation at Ser-544. |
Function | May play a role in the integrity of hemidesmosome and the attachment of basal keratinocytes to the underlying basement membrane. The 120 kDa linear IgA disease antigen is an anchoring filament component involved in dermal-epidermal cohesion. Is the target of linear IgA bullous dermatosis autoantibodies. |
Protein Name | Collagen Alpha-1(Xvii Chain180 Kda Bullous Pemphigoid Antigen 2Bullous Pemphigoid Antigen 2 Cleaved Into - 120 Kda Linear Iga Disease Antigen120 Kda Linear Iga Dermatosis AntigenLinear Iga Disease Antigen 1Lad-1 - 97 Kda Linear Iga Disease Antigen97 Kda Linear Iga Bullous Dermatosis Antigen97 Kda Lad Antigen97-LadLinear Iga Bullous Disease Antigen Of 97 KdaLabd97 |
Database Links | Reactome: R-HSA-1442490Reactome: R-HSA-1650814Reactome: R-HSA-198933Reactome: R-HSA-2022090Reactome: R-HSA-446107Reactome: R-HSA-8948216 |
Cellular Localisation | Cell JunctionHemidesmosomeMembraneSingle-Pass Type Ii Membrane ProteinLocalized Along The Plasma Membrane Of The Hemidesmosome120 Kda Linear Iga Disease Antigen: SecretedExtracellular SpaceExtracellular MatrixBasement MembraneExclusively Localized To Anchoring FilamentsLocalized To The Epidermal Side Of Split Skin97 Kda Linear Iga Disease Antigen: SecretedLocalized In The Lamina Lucida Beneath The Hemidesmosomes |
Alternative Protein Names | Collagen Alpha-1(Xvii Chain protein180 Kda Bullous Pemphigoid Antigen 2 proteinBullous Pemphigoid Antigen 2 Cleaved Into - 120 Kda Linear Iga Disease Antigen protein120 Kda Linear Iga Dermatosis Antigen proteinLinear Iga Disease Antigen 1 proteinLad-1 - 97 Kda Linear Iga Disease Antigen protein97 Kda Linear Iga Bullous Dermatosis Antigen protein97 Kda Lad Antigen protein97-Lad proteinLinear Iga Bullous Disease Antigen Of 97 Kda proteinLabd97 proteinCOL17A1 proteinBP180 proteinBPAG2 protein |
Information sourced from Uniprot.org