Product Name	ADAMTSL2
Chinese Name	整合素样金属蛋白酶与凝血酶样2蛋白抗体
Alias	ADAMTS like 2; ADAMTS like protein 2; ADAMTS-like protein 2; ADAMTSL 2; ADAMTSL-2; ADAMTSL2; ATL2_HUMAN.
Research Area	Tumour  Cardiovascular  Cell biology  immunology  Signal transduction  Growth factors and hormones  Cell adhesion molecule
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human,  (predicted: Mouse, Rat, )
Applications	WB=1:500-2000 ELISA=1:5000-10000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	102kDa
Cellular localization	Secretory protein
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human ADAMTSL2: 522-580/951
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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 Extracellular matrix蛋白
Product Picture	Sample: Lane 1: Human SH-SY5Y cell lysates Lane 2: Human HepG2 cell lysates Primary: Anti-ADAMTSL2 (SL5862R) 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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