| Tissue Specificity | Detected in skin. In the cornea, it is detected in the epithelial basement membrane, the epithelial cells, and at a lower level in stromal cells (at protein level). Stratified squamous epithelia. Found in hemidesmosomes. Expressed in cornea, oral mucosa, esophagus, intestine, kidney collecting ducts, ureter, bladder, urethra and thymus but is absent in lung, blood vessels, skeletal muscle and nerves. |
| 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 Antibody Names | Anti-Collagen Alpha-1(Xvii Chain antibodyAnti-180 Kda Bullous Pemphigoid Antigen 2 antibodyAnti-Bullous Pemphigoid Antigen 2 Cleaved Into - 120 Kda Linear Iga Disease Antigen antibodyAnti-120 Kda Linear Iga Dermatosis Antigen antibodyAnti-Linear Iga Disease Antigen 1 antibodyAnti-Lad-1 - 97 Kda Linear Iga Disease Antigen antibodyAnti-97 Kda Linear Iga Bullous Dermatosis Antigen antibodyAnti-97 Kda Lad Antigen antibodyAnti-97-Lad antibodyAnti-Linear Iga Bullous Disease Antigen Of 97 Kda antibodyAnti-Labd97 antibodyAnti-COL17A1 antibodyAnti-BP180 antibodyAnti-BPAG2 antibody |
Information sourced from Uniprot.org
