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ADAMTSL2 Rabbit pAb (bs-5862R)  
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产品编号 bs-5862R
英文名称 ADAMTSL2 Rabbit pAb
中文名称 整合素样金属蛋白酶与凝血酶样2蛋白抗体
别    名 ADAMTS like 2; ADAMTS like protein 2; ADAMTS-like protein 2; ADAMTSL 2; ADAMTSL-2; ADAMTSL2; ATL2_HUMAN.  
研究领域 肿瘤  心血管  细胞生物  免疫学  信号转导  生长因子和激素  细胞粘附分子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human (predicted: Mouse,Rat)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 102 kDa
检测分子量
细胞定位 分泌型蛋白 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ADAMTSL2: 522-580/951 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 ADAMTS (A Disintegrin And Metalloproteinase Domain with Thrombospondin type 1 Modules) is a family of zinc-dependent proteases that are implicated in a variety of normal and pathological conditions, including arthritis and cancer. ADAMTS protein family members contain an amino-terminal propeptide domain, a metalloproteinase domain, a disintegrin-like domain and a carboxy-terminus that contains a varying number of Thrombospondin type 1 (TSP-1) motifs. ADAMTS-L2 (ADAMTS-like protein 2) is a 951 amino acid secreted protein that is highly expressed in lung, kidney and liver. Mutations in the gene encoding ADAMTS are the cause of geleophysic dysplasia, an autosomal recessive disorder characterized by cardiac vavular anomalies, short stature, thick skin and brachydactyly. In individuals affected with geleophysic dysplasia, there is a significant increase in total active TGF-beta 1 and nuclear locations of p-SAMD2 in fibroblasts. Interestingly, ADAMTS-L2 interacts with LTBP-1, a glycoprotein that is part of the platelet-derived TGF-beta 1 complex.

Function:
Defects in ADAMTSL2 are the cause of geleophysic dysplasia [MIM:231050]. Geleophysic dysplasia is an autosomal recessive disorder characterized by short stature, brachydactyly, thick skin and cardiac valvular anomalies often responsible for an early death.

Subcellular Location:
Secreted.

Post-translational modifications:
Glycosylated (By similarity). Can be O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X(2)-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1,3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS family members. Also can be C-glycosylated with one or two mannose molecules on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and N-glycosylated. These other glycosylations can also facilitate secretion (By similarity).

DISEASE:
Defects in ADAMTSL2 are the cause of geleophysic dysplasia (GLPD) [MIM:231050]. Geleophysic dysplasia is an autosomal recessive disorder characterized by short stature, brachydactyly, thick skin and cardiac valvular anomalies often responsible for an early death.

Similarity:
Contains 1 PLAC domain.
Contains 7 TSP type-1 domains.

SWISS:
Q86TH1

Gene ID:
9719

Database links:

Entrez Gene: 9719 Human

Entrez Gene: 77794 Mouse

Omim: 612277 Human

SwissProt: Q86TH1 Human

SwissProt: Q7TSK7 Mouse

Unigene: 522543 Human

Unigene: 330088 Mouse



细胞外基质蛋白
产品图片
Sample: Lane 1: Human SH-SY5Y cell lysates Lane 2: Human HepG2 cell lysates Primary: Anti-ADAMTSL2 (bs-5862R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 102 kDa Observed band size: 135 kDa
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