ADAMTSL2,整合素样金属蛋白酶与凝血酶样2蛋白抗体

产品编号HZ-5862R
英文名称ADAMTSL2
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.
规格价格0.1ml 0.2ml 
说 明 书0.1ml 0.2ml
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, Mouse, Rat,
ADAMTSL2,整合素样金属蛋白酶与凝血酶样2蛋白抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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102kDa
细胞定位分泌型蛋白
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human ADAMTSL2
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.
ADAMTSL2,整合素样金属蛋白酶与凝血酶样2蛋白抗体PubMedPubMed
产品介绍background:
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).

ADAMTSL2,整合素样金属蛋白酶与凝血酶样2蛋白抗体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.

Database links:
UniProtKB/Swiss-Prot: Q86TH1.1

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.