CCM2 antibody

Catalog No. ABIN1589849

Product Details anti-CCM2 Antibody

Target: CCM2 (Cerebral Cavernous Malformation 2 (CCM2))

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This CCM2 antibody is un-conjugated

Application: Western Blotting (WB), Immunofluorescence (IF)

Specificity: Recombinant human CCM2

Characteristics: Chromosomal location: 7p13

Isotype: IgG

Application Details

Application Notes: Western Blot: use 1-5 µg/mL

Comment:

Rabbit IG Polyclonal Antibody

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Reconstitute in sterile water to a concentration of 0.1-1.0 mg/mL.

Buffer: 0.5X PBS, pH 7.2

Handling Advice: Centrifuge vial prior to opening. Avoid repeated freeze-thaw cycles.

Storage: -20 °C

Storage Comment: The lyophilized antibody is stable at room temperature for up to 1 month. The reconstituted antibody is stable for at least two weeks at 2-8 °C. Frozen aliquots are stable for at least 6 months when stored at -20 °C.

Expiry Date: 6 months

Target Details for CCM2

Target: CCM2 (Cerebral Cavernous Malformation 2 (CCM2))

Alternative Name: CCM-2 (CCM2 Products)

Synonyms: C7orf22 antibody, OSM antibody, malcavernin antibody, CCM2 antibody, BC029157 antibody, TUF2 antibody, vtn antibody, zgc:110233 antibody, CCM2 scaffolding protein antibody, cerebral cavernous malformation 2 antibody, malcavernin antibody, CCM2 antibody, Ccm2 antibody, LOC100304744 antibody, ccm2 antibody

Background: 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.
Synonyms: CCM-2, malcavernin, cerebral cavernous malformation 2, OSM, C7orf22, PP10187

NCBI Accession: NP_001025006, NM_001029835

UniProt: Q9BSQ5

Pathways: Cell-Cell Junction Organization