catalog number :
MBS9422124
products type :
Recombinant Protein
products full name :
Recombinant Human Collagen alpha-1 (XVII) chain protein
products short name :
[Collagen alpha-1 (XVII) chain]
products name syn :
[180 kDa bullous pemphigoid antigen 2; Bullous pemphigoid antigen 2]
other names :
[collagen alpha-1(XVII) chain; Collagen alpha-1(XVII) chain; collagen alpha-1(XVII) chain; collagen type XVII alpha 1 chain; 180 kDa bullous pemphigoid antigen 2; Bullous pemphigoid antigen 2]
products gene name :
[COHA1]
other gene names :
[COL17A1; COL17A1; ERED; BP180; BPA-2; BPAG2; LAD-1; BA16H23.2; BP180; BPAG2; LAD-1; 97 kDa LAD antigen; 97-LAD; LABD97]
sequence :
YLTSPDVRSFIVGPPGPPGPQGPPGDSRLLSTDASHSRG
SSSSSHSSSVRRGSSYSSSMSTGGGGAGSLGAGGAFGEA
AGDRGPYGTDIGPGGGYGAAAEGGMYAGNGGLLGADFAG
DLDYNELAVRVSESMQRQGLLQGMAYTVQGPPGQPGPQG
PPGISKVFSAYSNVTADLMDFFQTYGAIQGPPGQKGEMG
TPGPKGDRGPAGPPGHPGPPGPRGHKGEKGDKGDQVYAG
RRRRRSIAVKP
purity :
Greater than 90% as determined by SDS-PAGE.
form :
Tris-based buffer50% glycerol
storage stability :
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself. Generally, the shelf life of liquid form is 6 months at -20°C,-80°C. The shelf life of lyophilized form is 12 months at -20°C,-80°C. Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
other info1 :
Immunogen Description: Expression Region: 1253-1497aa Sequence Info: Partial. Calculated MW: 28.4 kDa. Tag Info: N-terminal 6xHis-tagged
products description :
May play a role in the integrity of hidesmosome and the attachment of basal keratinocytes to the underlying basent mbrane.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.
ncbi acc num :
NP_000485.3
ncbi gb acc num :
NM_000494.3
ncbi pathways :
Alpha6-Beta4 Integrin Signaling Pathway (198807); Cell Junction Organization Pathway (160966); Cell-Cell Communication Pathway (477132); Collagen Biosynthesis And Modifying Enzymes Pathway (645289); Collagen Degradation Pathway (730309); Collagen Formation Pathway (645288); Degradation Of The Extracellular Matrix Pathway (576263); Extracellular Matrix Organization Pathway (576262); Protein Digestion And Absorption Pathway (172847); Protein Digestion And Absorption Pathway (171868)
ncbi summary :
This gene encodes the alpha chain of type XVII collagen. 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. [provided by RefSeq, Jul 2008]
uniprot summary :
COL17A1: May play a role in the integrity of hemidesmosome and the attachment of basal keratinocytes to the underlying basement membrane. Defects in COL17A1 are a cause of generalized atrophic benign epidermolysis bullosa (GABEB). GABEB is a non- lethal, adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin, associated with hair and tooth abnormalities. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Extracellular matrix; Membrane protein, integral; Motility/polarity/chemotaxis. Chromosomal Location of Human Ortholog: 10q25.1. Cellular Component: endoplasmic reticulum lumen; extracellular region; hemidesmosome; integral to plasma membrane; intercellular junction; plasma membrane. Molecular Function: protein binding. Biological Process: cell-matrix adhesion; epidermis development; hemidesmosome assembly; regulation of immune response. Disease: Epidermolysis Bullosa, Junctional, Non-herlitz Type; Epithelial Recurrent Erosion Dystrophy