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Collagen XVII Recombinant Rabbit mAb (bsm-52041R)  
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產品編號 bsm-52041R
英文名稱 Collagen XVII Recombinant Rabbit mAb
中文名稱 膠原蛋白17重組兔單抗
別    名 Bullous Pemphigoid 180; 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.  XVII型膠原蛋白; 17型膠原蛋白
研究領域 細胞生物  信號轉導  
抗體來源 Rabbit
克隆類型 Recombinant
克 隆 號 1C11
交叉反應 Human,Mouse,Rat
產品應用 WB=1:500-2000,IHC-P=1:50-200,IHC-F=1:50-200,IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 150 kDa
檢測分子量
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/1ml
免 疫 原 A synthesized peptide derived from human Collagen XVII alpha 1: 1333-1370/1497 <Extracellular>
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

DISEASE:
Generalized atrophic benign epidermolysis bullosa (GABEB) [MIM:226650]: A non-lethal, adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin, associated with hair and tooth abnormalities. Note=The disease is caused by mutations affecting the gene represented in this entry.

SWISS:
Q9UMD9

Gene ID:
1308

Database links:

Entrez Gene: 1308 Human

Omim: 113811 Human

SwissProt: Q9UMD9 Human

Unigene: 117938 Human



產品圖片
25 ug total protein per lane of various lysates (see on figure) probed with Collagen XVII monoclonal antibody, unconjugated (bsm-52041R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
Paraformaldehyde-fixed, paraffin embedded Human Skin; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Collagen XVII Monoclonal Antibody, Unconjugated(bsm-52041R) at 1:200 overnight at 4°C, followed by conjugation to the SP Kit(Rabbit, SP-0023)and DAB (C-0010) staining.
(Negative control) Paraformaldehyde-fixed, paraffin embedded Rat Pancreas; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Collagen XVII Monoclonal Antibody, Unconjugated(bsm-52041R) at 1:200 overnight at 4°C, followed by conjugation to the SP Kit(Rabbit, SP-0023)and DAB (C-0010) staining.
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