Rabbit Anti-Human CCM-2
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| Cat-Nr. | 102-PA26 |
| Size | 200 µg |
| Price | 305 € |
| 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 CCM2 (RT #300-055) |
| Application | WB, IF |
| Synonyms | CCM-2; malcavernin; cerebral cavernous malformation 2; OSM; C7orf22; PP10187 |
| Description | Cerebral cavernous malformations (CCMs) are sporadically acquired or inherited vascular lesions of the central nervous system consisting of clusters of dilated thin-walled blood vessels that predispose individuals to seizures and stroke. Familial CCM is caused by mutations in KRIT1 (CCM1) or in malcavernin (CCM2). The roles of the CCM proteins in the pathogenesis of the disorder remain largely unknown. It was shown that the CCM1 gene product, KRIT1, interacts with the CCM2 gene product, malcavernin. Analogous to the established interactions of CCM1 and beta1 integrin with ICAP1, the CCM1/CCM2 association is dependent upon the phosphotyrosine binding (PTB) domain of CCM2. A familial CCM2 missense mutation abrogates the CCM1/CCM2 interaction, suggesting that loss of this interaction may be critical in CCM pathogenesis. CCM2 and ICAP1 bound to CCM1 via their respective PTB domains differentially influence the subcellular localization of CCM1. The data indicate that the genetic heterogeneity observed in familial CCM may reflect mutation of different molecular members of a coordinated signaling complex. |
| Uniprot ID | Q9BSQ5 |
| Protein RefSeq | NP_001025006.1 |
| mRNA RefSeq | NM_001029835.2 |
Figures
Immunofluorescence staining (green) of human foreskin (cryo-section of unfixed tissue) with anti-human CCM2 (K5546; dilution 1:50) [Cat# 102-PA26]. A) Note specific staining in the wall of microvessels. B) Negative control of a consecutive section. Note non-specific fluorescence in elastic fibres in the adventitia of an arteriol. 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 anti-human CCM-2.
The experiment was performed by Elisabetta Dejana’s group, IFOM-IEO-Campus, Milan Italy
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