Rabbit Anti-Human CCM-2

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Cat-Nr.102-PA26S
Size100 µg
Price175 €
CategoryPolyclonal Antibody
Clone Nr.Rabbit IG
Species ReactivityHuman
Formulationlyophilized
Buffer0.5X PBS, pH 7.2
ReconstitutionCentrifuge vial prior to opening. Reconstitute in sterile water to a concentration of 0.1-1.0 mg/ml.
Stability and StorageThe 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. Avoid repeated freeze-thaw cycles!
AntigenRecombinant human CCM2 (RT #300-055)
ApplicationWB, IF
SynonymsCCM-2; malcavernin; cerebral cavernous malformation 2; OSM; C7orf22; PP10187
DescriptionCerebral 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 IDQ9BSQ5
Protein RefSeqNP_001025006.1
mRNA RefSeqNM_001029835.2

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