• ATL2 rabbit pAb
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YP-Ab-08101-100UL

ATL2 rabbit pAb

ATL2 rabbit pAb

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  • Gene Name: ADAMTSL2 KIAA0605
  • Immunogen: Synthesized peptide derived from human ATL2 AA range: 194-244
  • Specificity: This antibody detects endogenous levels of ATL2 at Human/Mouse
  • Composition: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.216% sodium azide.
  • Source: Polyclonal, Rabbit,IgG
  • Dilution ratio: WB 1:500-2000
  • Purification process: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
  • Concentration: 1 mg/ml
  • Storage: -20°C/1 year
  • Other Names: ADAMTS-like protein 2 (ADAMTSL-2)
  • Background: This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) and ADAMTS-like protein family. Members of the family share several distinct protein modules, including a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. Individual members of this family differ in the number of C-terminal TS motifs, and some have unique C-terminal domains. The protein encoded by this gene lacks the protease domain, and is therefore of a member of the the ADAMTS-like protein subfamily. It is a secreted glycoprotein that binds the cell surface and extracellular matrix; it also interacts with latent transforming growth factor beta binding protein 1. Mutations in this gene have been associated with geleophysic dysplasia. [provided by RefSeq, Feb 2009],
  • Function: caution:Although strongly similar to members of the ADAMTS family it lacks the metalloprotease and disintegrin-like domains which are typical of that family.,disease: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.,miscellaneous:There is a significant increase in total and active TGFB1 in the culture medium as well as nuclear localization of phosphorylated SMAD2 in fibroblasts from individuals with geleophysic dysplasia.,similarity:Contains 1 PLAC domain.,similarity:Contains 7 TSP type-1 domains.,subunit:Interacts with LTBP1.,
  • Species: Human; Mouse
  • Range: WB
  • Protein: ATL2
  • DA: 105kD
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