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Collagen Type XVII (COL17)

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COL17A1; BP180; BPAG2; LAD-1; LABD97; Collagen Alpha-1(XVII)chain; 180 kDa bullous pemphigoid antigen 2; Bullous pemphigoid antigen 2; Linear IgA disease antigen 1

Collagen Type XVII (COL17)
CCC C135
HGNC 2194
MGI 88450
PubMed 10577906
RGD 1311130
UniProt Q9UMD9
Wiki COL17A1

Collagen XVII, previously called BP180, is a transmembrane protein which plays a critical role in maintaining the linkage between the intracellular and the extracellular structural elements involved in epidermal adhesion .Unlike most collagens, collagen XVII is a transmembrane protein. Collagen XVII is a structural component of hemidesmosomes, multiprotein complexes at the dermal-epidermal basement membrane zone that mediate adhesion of keratinocytes to the underlying membrane. Mutations in this gene are associated with both generalized atrophic benign and junctional epidermolysis bullosa. Two homotrimeric forms of type XVII collagen exist. The full length form is the transmembrane protein. A soluble form, referred to as either ectodomain or LAD-1, is generated by proteolytic processing of the full length form.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPC135Hu01 Recombinant Collagen Type XVII (COL17) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAC135Hu01 Polyclonal Antibody to Collagen Type XVII (COL17) WB; IHC; ICC; IP.
MAC135Hu22 Monoclonal Antibody to Collagen Type XVII (COL17) WB; IHC; ICC; IP.
LAC135Hu71 Biotin-Linked Polyclonal Antibody to Collagen Type XVII (COL17) WB; IHC; ICC.
Assay Kits SEC135Hu ELISA Kit for Collagen Type XVII (COL17) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Collagen Type XVII (COL17) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Collagen Type XVII (COL17) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Collagen Type XVII (COL17) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Collagen Type XVII (COL17) CLIA Kit Customized Service Offer
n/a ELISA Kit for Collagen Type XVII (COL17) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Collagen Type XVII (COL17) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Collagen Type XVII (COL17) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Collagen Type XVII (COL17) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Collagen Type XVII (COL17) CLIA Kit Customized Service Offer
n/a ELISA Kit for Collagen Type XVII (COL17) ELISA Kit Customized Service Offer
  1. "Cloning and primary structural analysis of the bullous pemphigoid autoantigen, BP180."J. Invest. Dermatol. 99:243-250(1992) [PubMed] [Europe PMC] [Abstract]
  2. "Cloning of the human type XVII collagen gene (COL17A1), and detection of novel mutations in generalized atrophic benign epidermolysis bullosa."Am. J. Hum. Genet. 60:352-365(1997) [PubMed] [Europe PMC] [Abstract]
  3. "The DNA sequence and comparative analysis of human chromosome 10." Nature 429:375-381(2004) [PubMed] [Europe PMC] [Abstract]
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  5. "Genomic organization of collagenous domains and chromosomal assignment of human 180-kDa bullous pemphigoid antigen-2, a novel collagen of stratified squamous epithelium."J. Biol. Chem. 266:24064-24069(1991) [PubMed] [Europe PMC] [Abstract]
  6. "The 97-kDa (LABD97) and 120-kDa (LAD-1) fragments of bullous pemphigoid antigen 180/type XVII collagen have different N-termini."J. Invest. Dermatol. 121:1554-1556(2003) [PubMed] [Europe PMC] [Abstract]
  7. "The 97 kDa linear IgA bullous disease antigen is identical to a portion of the extracellular domain of the 180 kDa bullous pemphigoid antigen, BPAg2."J. Invest. Dermatol. 110:207-210(1998) [PubMed] [Europe PMC] [Abstract]
  8. "LAD-1, the linear IgA bullous dermatosis autoantigen, is a novel 120-kDa anchoring filament protein synthesized by epidermal cells."J. Invest. Dermatol. 106:734-738(1996) [PubMed] [Europe PMC] [Abstract]
  9. ErratumJ. Invest. Dermatol. 106:1343-1343(1996)
  10. "97-kDa linear IgA bullous dermatosis (LAD) antigen localizes to the lamina lucida of the epidermal basement membrane."J. Invest. Dermatol. 106:739-743(1996) [PubMed] [Europe PMC] [Abstract]
  11. "Evidence that the 180-kD bullous pemphigoid antigen is a transmembrane collagen, type XVII, in a triple-helical conformation and in type II transmembrane topography."J. Invest. Dermatol. 106:860-860(1996)
  12. "Two forms of collagen XVII in keratinocytes. A full-length transmembrane protein and a soluble ectodomain."J. Biol. Chem. 273:25937-25943(1998) [PubMed] [Europe PMC] [Abstract]
  13. "The N terminus of the transmembrane protein BP180 interacts with the N-terminal domain of BP230, thereby mediating keratin cytoskeleton anchorage to the cell surface at the site of the hemidesmosome."Mol. Biol. Cell 11:277-286(2000) [PubMed] [Europe PMC] [Abstract]
  14. "Transmembrane collagen XVII, an epithelial adhesion protein, is shed from the cell surface by ADAMs."EMBO J. 21:5026-5035(2002) [PubMed] [Europe PMC] [Abstract]
  15. "Analysis of the interactions between BP180, BP230, plectin and the integrin alpha6beta4 important for hemidesmosome assembly."J. Cell Sci. 116:387-399(2003) [PubMed] [Europe PMC] [Abstract]
  16. "Extracellular phosphorylation of collagen XVII by ecto-casein kinase 2 inhibits ectodomain shedding."J. Biol. Chem. 282:22737-22746(2007) [PubMed] [Europe PMC] [Abstract]
  17. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  18. "Compound heterozygosity for a dominant glycine substitution and a recessive internal duplication mutation in the type XVII collagen gene results in junctional epidermolysis bullosa and abnormal dentition."Am. J. Pathol. 148:1787-1796(1996) [PubMed] [Europe PMC] [Abstract]
  19. "Three novel homozygous point mutations and a new polymorphism in the COL17A1 gene: relation to biological and clinical phenotypes of junctional epidermolysis bullosa."Am. J. Hum. Genet. 60:1344-1353(1997) [PubMed] [Europe PMC] [Abstract]
  20. "Collagen XVII is destabilized by a glycine substitution mutation in the cell adhesion domain Col15."J. Biol. Chem. 275:3093-3099(2000) [PubMed] [Europe PMC] [Abstract]
  21. "Hemizygosity for a glycine substitution in collagen XVII: unfolding and degradation of the ectodomain."J. Invest. Dermatol. 115:207-212(2000) [PubMed] [Europe PMC] [Abstract]
  22. "A novel homozygous point mutation in the COL17A1 gene in a Chinese family with generalized atrophic benign epidermolysis bullosa."J. Dermatol. Sci. 28:181-186(2002) [PubMed] [Europe PMC] [Abstract]