XVII型胶原α-1链 (180 kDa大疱性类天疱疮抗原2) (大疱性类天疱疮抗原2) [裂解为: 120 kDa线性IgA病抗原 (120 kDa线性IgA皮肤病抗原) (线性IgA病抗原1) (LAD-1); 97 kDa线性IgA病抗原 (97 kDa线性IgA大疱性皮肤病抗原) (97 kDa LAD抗原) (97-LAD) (97 kDa线性IgA大疱病抗原) (LABD97)]
XVII型胶原α-1链 (180 kDa大疱性类天疱疮抗原2) (大疱性类天疱疮抗原2) [裂解为: 120 kDa线性IgA病抗原 (120 kDa线性IgA皮肤病抗原) (线性IgA病抗原1) (LAD-1); 97 kDa线性IgA病抗原 (97 kDa线性IgA大疱性皮肤病抗原) (97 kDa LAD抗原) (97-LAD) (97 kDa线性IgA大疱病抗原) (LABD97)]
Q9UMD9功能描述
可能在半桥粒的完整性及基底角质形成细胞与下方基底膜的附着中发挥作用。; FUNCTION: 120 kDa线性IgA病抗原是一种参与真皮-表皮粘连的锚丝成分。是线性IgA大疱性皮肤病自身抗体的靶标。
组织特异性
Detected in skin (PubMed:8618013). 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) (PubMed:25676728). 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.
亚细胞定位
Cell junction, hemidesmosome. Membrane; Single-pass type II membrane protein. Note=Localized along the plasma membrane of the hemidesmosome.; SUBCELLULAR LOCATION: [120 kDa linear IgA disease antigen]: Secreted, extracellular space, extracellular matrix, basement membrane. Note=Exclusively localized to anchoring filaments. Localized to the epidermal side of split skin.; SUBCELLULAR LOCATION: [97 kDa linear IgA disease antigen]: Secreted, extracellular space, extracellular matrix, basement membrane. Note=Localized in the lamina lucida beneath the hemidesmosomes.
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