CCM2 Rabbit Polyclonal Antibody

CAT#: AP26022PU-L

CCM2 rabbit polyclonal antibody, Aff - Purified

Product Images

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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  Immunofluorescence staining (green) of Human foreskin (cryo-section of unfixed tissue) with anti-Human CCM2 antibody. (K5546, dilution1/50). 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 anarteriol. Nuclei counter-stained with Dapi (blue). Specimen provided by Prof. Dr. J. Wilting, Goettingen. The experiment was performed by the research group of Prof. Dr. J. Wilting, University Göttingen, Germany.

Specifications

Product Data

• Applications: IF, WB
• Recommended Dilution: Western blot: 1-5 μg/ml.
  Immunofluorescence: 1/200.
  Immunohistochemistry.
• Reactivities: 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:

  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.

• Stability: Shelf life: one year from despatch.

• 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