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Anti-Collagen XVII Antibody (CAB4808)

SKU:
CAB4808
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology
€139 - €419
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Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-Collagen XVII Antibody
Antibody SKU:CAB4808
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC
Reactivity:Human
Host Species:Rabbit
Immunogen:A synthesized peptide derived from human Collagen XVII
Application:WB IHC
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:Human
Positive Samples:A-431
Immunogen:A synthesized peptide derived from human Collagen XVII
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:1308
Uniprot:Q9UMD9
Cellular Location:
Calculated MW:130, 180kDa
Observed MW:180KDa
Synonyms:BA16H23.2, BP180, BPA-2, BPAG2, ERED, LAD-1
Background: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 Protein Function: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.
UniProt Protein Details:

Protein type:Membrane protein, integral; Motility/polarity/chemotaxis; Extracellular matrix

Chromosomal Location of Human Ortholog: 10q24.3

Cellular Component: collagen; hemidesmosome; endoplasmic reticulum lumen; integral to plasma membrane; plasma membrane; extracellular region; basement membrane; intercellular junction

Molecular Function:protein binding

Biological Process: collagen catabolic process; extracellular matrix disassembly; extracellular matrix organization and biogenesis; epidermis development; hemidesmosome assembly; cell-matrix adhesion

Disease: Epidermolysis Bullosa, Junctional, Non-herlitz Type

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 Code:Q9UMD9
NCBI GenInfo Identifier:119829187
NCBI Gene ID:1308
NCBI Accession:NP_000485.3
UniProt Secondary Accession:Q9UMD9,Q02802, Q5JV36, Q99018, Q9NQK9, Q9UC14,
UniProt Related Accession:Q9UMD9
Molecular Weight:150,419 Da
NCBI Full Name:collagen alpha-1(XVII) chain
NCBI Synonym Full Names:collagen, type XVII, alpha 1
NCBI Official Symbol:COL17A1  
NCBI Official Synonym Symbols:BP180; BPA-2; BPAG2; LAD-1; BA16H23.2  
NCBI Protein Information:collagen alpha-1(XVII) chain; collagen alpha-1(XVII) chain; alpha 1 type XVII collagen; type XVII collagen alpha-1; collagen XVII, alpha-1 polypeptide; 180 kDa bullous pemphigoid antigen 2; bullous pemphigoid antigen 2 (180kD); bA16H23.2 (collagen, type X
UniProt Protein Name:Collagen alpha-1(XVII) chain
UniProt Synonym Protein Names:180 kDa bullous pemphigoid antigen 2; Bullous pemphigoid antigen 2Cleaved into the following 2 chains:120 kDa linear IgA disease antigen; Alternative name(s):; 120 kDa linear IgA dermatosis antigen; Linear IgA disease antigen 1; LAD-197 kDa linear IgA disease antigen; Alternative name(s):; 97 kDa linear IgA bullous dermatosis antigen; 97 kDa LAD antigen; 97-LAD; Linear IgA bullous disease antigen of 97 kDa
UniProt Gene Name:COL17A1  
UniProt Entry Name:COHA1_HUMAN
Anti-Collagen XVII Antibody (CAB4808)Western blot - Collagen XVII Rabbit mAb (CAB4808)

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