KRIT1 Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IF, WB
Recommend Dilution	Western Blot: 1-5 μg/ml. Immunofluorescence: 1-10 μg/ml. Immunohistochemistry: 1/200.
Reactivity	Human
Host	Rabbit
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.
Isotype	IgG
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
Conjugation	Unconjugated
Storage Condition	Prior to reconstitution store at 2-8°C. 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	KRIT1, ankyrin repeat containing
Database Link	Entrez Gene 889 Human UniProt ID O00522
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

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