| 產(chǎn)品編號 | bs-5862R |
| 英文名稱 | ADAMTSL2 Rabbit pAb |
| 中文名稱 | 整合素樣金屬蛋白酶與凝血酶樣2蛋白抗體 |
| 別 名 | ADAMTS like 2; ADAMTS like protein 2; ADAMTS-like protein 2; ADAMTSL 2; ADAMTSL-2; ADAMTSL2; ATL2_HUMAN. |
| 研究領(lǐng)域 | 腫瘤 心血管 細(xì)胞生物 免疫學(xué) 信號轉(zhuǎn)導(dǎo) 生長因子和激素 細(xì)胞粘附分子 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human (predicted: Mouse,Rat) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 102 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 分泌型蛋白 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ADAMTSL2: 522-580/951 |
| 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(chǎn)品介紹 |
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 細(xì)胞外基質(zhì)蛋白 |
| 產(chǎn)品圖片 |
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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