CCM2 Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IF, WB
Recommend Dilution	Western blot: 1-5 μg/ml. Immunofluorescence: 1/200. Immunohistochemistry.
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	Highly pure (> 95%) recombinant Human CCM-2 (Cerebral cavernous malformations 2 protein; aa: Met1-Ala444) from E.coli (Cat.-No AR26003PU-N).
Specificity	This antibody detects recombinant Human CCM-2 in Western Blot and native CCM-2 in Immunohistochemistry.
Formulation	5mM PBS pH 7.2 without preservatives State: Aff - Purified State: Lyophilized purified Ig fraction
Reconstitution Method	Restore in sterile water to a concentration of 0.1-1.0 mg/ml.
Purification	Protein A Chromatography
Conjugation	Unconjugated
Storage Condition	Prior to reconstitution store at 2-8°C for one month or dessicated at -20°C for longer. Following reconstitution store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Gene Name	CCM2 scaffolding protein
Database Link	Entrez Gene 83605 Human UniProt ID Q9BSQ5
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	CCM2, C7orf22, PP10187
Reference Data

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