Rabbit Anti-Human CCM-1

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Cat-Nr.102-PA25
Size200 µg
Price290 €
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 CCM1 (RT #300-054)
ApplicationWB, IF
SynonymsCCM-1; Cerebral cavernous malformations protein 1; KRIT1; KRIT1, ankyrin repeat containing; CAM
DescriptionCerebral 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.
Uniprot IDO00522
Protein RefSeqNP_004903.2
mRNA RefSeqNM_004912,3

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