Description
Product Usage Information
Western Blotting: 1:1000
Simple Western™: 1:10 - 1:50
Immunoprecipitation: 1:50
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.
Protocol
Available protocols: Western Blotting, Immunoprecipitation
Specificity / Sensitivity
CCM2 (F8G7Z) Rabbit Monoclonal Antibody recognizes endogenous levels of total CCM2 protein.
Species Reactivity: Human, Mouse, Rat, Monkey
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Gln100 of human CCM2 protein.
Background
The cerebral cavernous malformation (CCM) proteins CCM1/KRIT1, CCM2/Malcavernin, and CCM3/PDCD10 form a heterotrimeric complex critical for stabilizing endothelial cell junctions and thus blood vessel formation and vascular integrity (1). Loss of function mutations of any of the three complex members is associated with formation of CCMs, characterized by weak, inelastic, and leaky epithelium that can lead to seizures, stroke, and other neurological symptoms (2-5). CCM2 contains an N-terminal phosphotyrosine binding domain (PTB) that interacts with TrkA receptor (6), where it induces cell death in medulloblastoma cells after activation by STK25 kinase (7). CCM2 C-terminal harmonin homology domain (HHD) binds and suppresses the activity of MEKK3 kinase, and increased expression of MEKK3 target genes is observed in early CCM lesions (8). Gain-of-function mutations in PI3 Kinase are observed in the majority of CCM lesions (9), and these mutations synergize with CCM protein loss to increase mTOR signaling (9,10), suggesting that mTOR inhibitors may be a novel approach to treating CCM lesions.
Alternate Names
C7orf22; CCM2; CCM2 scaffold protein; CCM2 scaffolding protein; cerebral cavernous malformation 2; Cerebral cavernous malformations 2 protein; Malcavernin; MGC4067; MGC4607; MGC74868; OSM; osmosensing scaffold for MEKK3; PP10187
