Von Hippel-Lindau syndrome (VHL) is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign tumors. A germline mutation of VHL gene is the basis of familial inheritance of VHL syndrome. The protein is a component of the protein complex that includes elongin B, elongin C, and cullin-2, and possesses ubiquitin ligase E3 activity. This protein is involved in the ubiquitination and degradation of hypoxia-inducible-factor (HIF), which is a transcription factor that plays a central role in the regulation of gene expression by oxygen. RNA polymerase II subunit POLR2G/RPB7 is also reported to be a target of this protein.
应用类型
WB, IHC-P, Flow
免疫原
This VHL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 181-210 amino acids from the C-terminal region of human VHL.
来源宿主
兔
反应性
人
保存建议
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.