| ELK.No | ES18200 |
| Product name | ATL2 rabbit pAb |
| Reactivity | Human; Mouse |
| Applications | WB |
| Other name | ADAMTS-like protein 2 (ADAMTSL-2) |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 9719 |
| Human Swiss-Prot | Q86TH1 |
| Source | Rabbit |
| Isotype | IgG |
| Target | ATL2 |
| Fields | |
| Gene name | ADAMTSL2 KIAA0605 |
| Protein name | ATL2 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 77794 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q7TSK7 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| Immunogen | Synthesized peptide derived from human ATL2 AA range: 194-244 |
| Specificity | This antibody detects endogenous levels of ATL2 at Human/Mouse |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.216% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | |
| Observed band (KD) | 105kD |
| 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., |
| Subcellular location | Secreted . |
| Expression | Brain,PNS, |

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