Anti-Collagen XVII抗体This gene encodes the alpha chain of type XVII collagen. Unlike most collagens, collagen XVII is a transmembrane protein.

产品编号 yb-13964R
英文名称 Anti-Collagen XVII抗体
中文名称 XVII型胶原蛋白/胶原蛋白17/17型胶原蛋白抗体
别    名 180 kDa bullous pemphigoid antigen 2; Alpha 1 type XVII collagen; BA16H23.2; BP 180; BP180; BPAG 2; BPAG2; Bullous pemphigoid antigen 2; COL17A1; Collagen 17; Collagen alpha 1 XVII chain; Collagen alpha 1(XVII) chain; Collagen alpha1 XVII chain; Collagen type XVII alpha 1; Collagen XVII alpha 1 polypeptide; CollagenXVII; Epidermolysis bullosa junctional localisata variant; FLJ60881; KIAA0204; LAD 1; LAD.
Anti-Collagen XVII抗体
说 明 书 0.1ml  0.2ml  
研究领域 细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 150kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human Collagen XVII
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
产品介绍 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]

Function:
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 the gene coding for collagen XVII 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. Two transcript variants, one resulting from alternative splicing in the 3' UTR, have been identified for this gene.

Subunit:
Homotrimers of alpha 1(XVII)chains. Interacts (via cytoplasmic region) with ITGB4 (via cytoplasmic region). Interacts (via cytoplasmic region) with DST isoform 3 (via N-terminus). Interacts (via N-terminus) with PLEC. Interacts (via cytoplasmic region) with DSP.

Subcellular Location:
Cell junction, hemidesmosome. Membrane; Single-pass type II membrane protein. Note=Localized along the plasma membrane of the hemidesmosome. 120 kDa linear IgA disease antigen and 97 kDa linear IgA disease antigen: Secreted, extracellular space, extracellular matrix, basement membrane.

Post-translational modifications:
The intracellular/endo domain is disulfide-linked.