Rabbit Anti-Human CCM-1
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| Cat-Nr. | 102-PA25S |
| Size | 100 µg |
| Price | 185 € |
| Category | Polyclonal Antibody |
| Clone Nr. | Rabbit IgG |
| Species Reactivity | Human |
| Formulation | lyophilized |
| Buffer | 0.5X PBS, pH 7.2 |
| Reconstitution | Centrifuge vial prior to opening. Reconstitute in sterile water to a concentration of 0.1-1.0 mg/ml. |
| Stability and Storage | The lyophilized antibody is stable for at least 2 years at -20°C. After sterile reconstitution the antibody is stable at 2-8°C for up to 6 months. Frozen aliquots are stable for at least 6 months when stored at -20°C. Addition of a carrier protein or 50% glycerol is recommended for frozen aliquots. |
| Antigen | Recombinant human CCM1 (RT #300-054) |
| Application | WB, IF |
| Synonyms | CCM-1; Cerebral cavernous malformations protein 1; KRIT1; KRIT1, ankyrin repeat containing; CAM |
| Description | Cerebral cavernous malformations (CCM) are frequent vascular abnormalities caused by mutations in one of the CCM genes. CCM-1 (also known as KRIT1) stabilizes endothelial junctions and is essential for vascular morphogenesis in mouse embryos. However, cellular functions of CCM-1 during the early steps of the CCM pathogenesis remain unknown. It was shown that CCM-1 represents an antiangiogenic protein to keep the human endothelium quiescent. CCM-1 inhibits endothelial proliferation, apoptosis, migration, lumen formation, and sprouting angiogenesis in primary human endothelial cells. CCM-1 strongly induces DLL4-NOTCH signaling, which promotes AKT phosphorylation but reduces phosphorylation of the mitogen-activated protein kinase ERK. Consistently, blocking of NOTCH activity alleviates CCM-1 effects. ERK phosphorylation is increased in human CCM lesions. Transplantation of CCM-1-silenced human endothelial cells into SCID mice recapitulates hallmarks of the CCM pathology and serves as a unique CCM model system. |
| Uniprot ID | O00522 |
| Protein RefSeq | NP_004903.2 |
| mRNA RefSeq | NM_004912,3 |
Figures
Immunofluorescence staining of human foreskin (cryo-section of unfixed tissue) with anti-CCM1 (red; dilution 1:50) [Cat# 102-PA25]. Costaining of endothelial cells with anti-CD31 (green). Note specific staining in the wall of a subset of vessel. Nuclei counter-stained with Dapi (blue).
The experiment was performed by the research group of Prof. Dr. J. Wilting, University Göttingen, Germany.
Western Blot analysis of recombinant human CCM-1 (FERM domain) and recombinant human full length CCM-1 using a rabbit polyclonal anti-human CCM-1 antibody [Cat# 102-PA25] generated against the recombinant FERM domain of human CCM-1.
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