Recombinant Human Amino-terminal enhancer of split/AES Protein (RMPP-00230802)
Cat. No.: RMPP-00230802
Category: Kinases
Research Area: Signal Transduction
INQUIRY
50 μg
250 µg
Product Features
| Source | Mammalian |
|---|---|
| Purity | > 95% SDS-PAGE. |
| Nature | Recombinant |
| Endotoxin Level | < 1.000 Eu/µg |
| Animal Free | No |
| Tags | His tag C-Terminus, Fc tag C-Terminus |
| Form | Lyophilized |
| Applications | SDS-PAGE; HPLC |
| Key Features | Expression system: Mammalian; Purity: > 95% SDS-PAGE; Endotoxin level: < 1.000 Eu/µg; Tags: His tag C-Terminus, Fc tag C-Terminus; Suitable for: SDS-PAGE, HPLC |
Protein Information
| UniProt ID | Q13873 |
|---|---|
| Molecular Weight | 42 kDa including tags |
| Sequence | SQNQERLCAFKDPYQQDLGIGESRISHENGTILCSKGSTCYGLWEKSKGD INLVKQGCWSHIGDPQECHYEECVVTTTPPSIQNGTYRFCCCSTDLCNVN FTENFPPPDTTPLSPPHSFNRDETIVDDIEGRMDEPKSCDKTHTCPPCPA PELLGGPSVFLFPPKPKDTLMISRTPEVTCVVVDVSHEDPEVKFNWYVDG VEVHNAKTKPREEQYNSTYRVVSVLTVLHQDWLNGKEYKCKVSNKALPAP IEKTISKAKGQPREPQVYTLPPSREEMTKNQVSLTCLVKGFYPSDIAVEW ESNGQPENNYKTTPPVLDSDGSFFLYSKLTVDKSRWQQGNVFSCSVMHEA LHNHYTQKSLSLSPGKHHHHHH |
| Sequence Similarities | Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily. Contains 1 protein kinase domain. |
| Protein Length | Protein fragment |
| Cellular Localization | Membrane. |
| Tissue Specificity | Highly expressed in heart and liver. |
| Function | On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Binds to BMP-7, BMP-2 and, less efficiently, BMP-4. Binding is weak but enhanced by the presence of type I receptors for BMPs. |
| Involvement in Disease | Defects in BMPR2 are the cause of primary pulmonary hypertension (PPH1). PPH1 is a rare autosomal dominant disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial PPH1 is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.Defects in BMPR2 are a cause of pulmonary venoocclusive disease (PVOD). PVOD is a rare form of pulmonary hypertension in which the vascular changes originate in the small pulmonary veins and venules. The pathogenesis is unknown and any link with PPH1 has been speculative. The finding of PVOD associated with a BMPR2 mutation reveals a possible pathogenetic connection with PPH1. |
Storage & Shipping
| Shipping and Storage | Shipped at 4°C. The lyophilized protein is stable for a few weeks at room temperature. Store at -20°C or -80°C. Avoid freeze / thaw cycle. pH: 7.40Constituent: 100% PBS0.2 µM filtered solution. |
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For research use only. Not for clinical use.
