货品编码： 博奥森.bs-7883R 品牌：博奥森 品名：Rabbit Anti-VHLH antibody (试用装) 规格： 10ul

产品描述： 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 this gene is the basis of familial inheritance of VHL syndrome.

The protein encoded by this gene 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.

Alternatively spliced transcript variants encoding distinct isoforms have been observed. [provided by RefSeq, Jul 2008].

Function: Involved in the ubiquitination and subsequent proteasomal degradation via the von Hippel-Lindau ubiquitination complex.

Seems to act as target recruitment subunit in the E3 ubiquitin ligase complex and recruits hydroxylated hypoxia-inducible

factor (HIF) under normoxic conditions. Involved in transcriptional repression through interaction with HIF1A, HIF1AN and

histone deacetylases. Ubiquitinates, in an oxygen-responsive manner, ADRB2.