Primary Information |
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| BoMiProt ID | Bomi8334 |
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| Protein Name | Protein FAM204A |
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| Organism | Bos taurus |
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| Uniprot ID | Q3ZCI6 |
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| Milk Fraction | Whey |
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| Ref Sequence ID | NP_001030224.1 |
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| Aminoacid Length | 233 |
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| Molecular Weight | 26722 |
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| FASTA Sequence |
Download |
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| Gene Name | FAM204A |
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| Gene ID | 507725 |
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| Protein Existence Status | reviewed |
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Secondary Information |
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| Protein Function | BP180 is a type II collagenous transmembrane protein and is best known as the major autoantigen in the blistering skin disease bullous pemphigoid (BP). The BP180 trimer is a central component in type I hemidesmosomes (HD), which cause the adhesion between epidermal keratinocytes and the basal lamina |
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| Biochemical Properties | Human BP180 (1497 aa) contains 15 collagenous domains that are flanked and interrupted by NC sequences in its extracellular domain (ECD, aa 489–497). The collagenous domains harbor the repeating triplet glycine-X-Y, where X is often proline and Y hydroxyproline. Mouse BP180 has a similar structure, with either 13 or 14 collagenous domains in each of its two transcriptional variants.The interspersed NC domains are denominated from the carboxy terminus as NC1, NC2 up to NC16 (in human BP180). The NC domain closest to the amino terminus (human NC16, mouse NC14) consists of an extracellular juxtamembrane region, (NC16A; human aa 489–566), a transmembrane domain (NC16B; human aa 468–488), and an intracellular domain. |
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| PTMs | Disulfide bond, Glycosylation, Hydroxylation at Pro |
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Site(s) of PTM(s)
N-glycosylation,
O-glycosylation,
Phosphorylation
| NA |
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| Predicted Disorder Regions | (1-233) |
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| DisProt Annotation | |
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| TM Helix Prediction | no TM helices |
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| Significance of PTMs | The intracellular/endo domain is disulfide-linked. |
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| Bibliography | 1.Tuusa J, Kokkonen N, Tasanen K. BP180/Collagen XVII: A Molecular View. Int J Mol Sci. 2021 Nov 12;22(22):12233. doi: 10.3390/ijms222212233. PMID: 34830116; PMCID: PMC8623354. 2.Cloning and primary structural analysis of the bullous pemphigoid autoantigen BP180.Giudice GJ, Emery DJ, Diaz LA J Invest Dermatol. 1992 Sep; 99(3):243-50. 3.Collagen structure: the Madras triple helix and the current scenario.Bhattacharjee A, Bansal M IUBMB Life. 2005 Mar; 57(3):161-72. 4.Cloning of type XVII collagen. Complementary and genomic DNA sequences of mouse 180-kilodalton bullous pemphigoid antigen (BPAG2) predict an interrupted collagenous domain, a transmembrane segment, and unusual features in the 5'-end of the gene and the 3'-untranslated region of the mRNA.Li K, Tamai K, Tan EM, Uitto J J Biol Chem. 1993 Apr 25; 268(12):8825-34. 5.Repetitive immunization breaks tolerance to type XVII collagen and leads to bullous pemphigoid in mice.Hirose M, Recke A, Beckmann T, Shimizu A, Ishiko A, Bieber K, Westermann J, Zillikens D, Schmidt E, Ludwig RJ J Immunol. 2011 Aug 1; 187(3):1176-83. |
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