KRIT1 Rabbit Polyclonal Antibody

CAT#: AP26021PU-L

KRIT1 rabbit polyclonal antibody, Purified

Product Images

Western analysis of recombinant Human CCM-1 (FERM domain) and recombinant Human full length CCM-1 using a Rabbit polyclonal anti-Human CCM-1 antibody generated against the recombinant FERM domain of Human CCM-1.

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  Immunofluorescence staining of Human foreskin (Cryo-section of unfixed tissue) with anti-CCM1 Antibody (red, dilution: 1/50). Costaining of endothelial cells with anti-CD31 (green). Note specific staining in the wall of a subset of vessel. Nuclei counter-stained with Dapi (blue). Specimen provided by Prof. Dr. J. Wilting and Dr. K. Buttler, Goettingen.

Specifications

Product Data

• Applications: IF, WB
• Recommended Dilution: Western Blot: 1-5 μg/ml.
  Immunofluorescence: 1-10 μg/ml.
  Immunohistochemistry: 1/200.
• Reactivities: Human
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: Highly pure (>95%) recombinant Human CCM-1 (Met1-Ser736) derived from E. coli fused to a C-teminal His-tag (6 x His) (Cat.-No AR26002PU-N).
• Specificity: This antibody is anti-His depleated.
  It detects KRIT1 / CCM1.
• Formulation: 5mM PBS, pH 7.2
  State: Purified
  State: Lyophilized purified IgG fraction
• Reconstitution Method: Restore in sterile water to a concentration of 0.1-1.0 mg/ml.
  Centrifuge vial prior to opening.
• Purification: Protein A Chromatography
• Gene Name: Homo sapiens KRIT1, ankyrin repeat containing (KRIT1), transcript variant 2
• Database Link:
  Entrez Gene 889 Human
• Background: Cerebral Cavernous Malformations (CCM) are frequent vascular abnormalities caused by mutations in one of the CCM genes. CCM-1 (also known as KRIT1) stabilizes endothelial junctions and is essential for vascular morphogenesis in mouse embryos. However, cellular functions of CCM-1 during the early steps of the CCM pathogenesis remain unknown. It was shown that CCM-1 represents an antiangiogenic protein to keep the human endothelium quiescent. CCM-1 inhibits endothelial proliferation, apoptosis, migration, lumen formation, and sprouting angiogenesis in primary human endothelial cells. CCM-1 strongly induces DLL4-NOTCH signaling, which promotes AKT phosphorylation but reduces phosphorylation of the mitogen-activated protein kinase ERK. Consistently, blocking of NOTCH activity alleviates CCM-1 effects. ERK phosphorylation is increased in human CCM lesions. Transplantation of CCM-1-silenced human endothelial cells into SCID mice recapitulates hallmarks of the CCM pathology and serves as a unique CCM model system.
• Synonyms: Krev interaction trapped 1

Reference Data

• Protein Families: Druggable Genome