ADAMTSL2 rabbit pAb
Ucallm_WP3623-50
Price range: $268.00 through $328.00
Product details
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],
Specifications
| Size | 50μL/100μL |
| Target | ADAMTSL2 |
| Reactivity | Human, Mouse |
| Applications | WB |
| MW(Calculated) | |
| MW(Observed) | 105kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-2000 |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | Synthesized peptide derived from human ATL2 AA range: 194-244 |
| Specificity | This antibody detects endogenous levels of ATL2 at Human/Mouse |
| Gene Name | ADAMTSL2 KIAA0605 |
| Protein Name | ADAMTS-like protein 2 |
| Other Name | ADAMTS-like protein 2; / ADAMTSL-2 |
| Species | Human |
| Gene ID | 9719 |
| Uniprot | Q86TH1, |
| Species.1 | Mouse |
| Gene ID.1 | 77794 |
| Uniprot.1 | Q7TSK7 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Secreted . |
| Tissue Expression | Brain,PNS, |
| Signaling_pathway | |
| Research Areas | |
| 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., |
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