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von Hippel-Lindau disease tumor suppressor

Definition:

Involved in the ubiquitination and subsequent proteasomal degradation via the von Hippel-Lindau ubiquitination complex. Seems to act as a 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.

References:

[1]. Jinke Cheng, et al. SUMO-specific protease 1 is essential for stabilization of HIF1alpha during hypoxia. Cell. 2007 Nov 2;131(3):584-95. [Content Brief]

[2]. Liang Xie, et al. Oxygen-regulated beta(2)-adrenergic receptor hydroxylation by EGLN3 and ubiquitylation by pVHL. Sci Signal. 2009 Jul 7;2(78):ra33. [Content Brief]

[3]. K Tanimoto, et al. Mechanism of regulation of the hypoxia-inducible factor-1 alpha by the von Hippel-Lindau tumor suppressor protein. EMBO J. 2000 Aug 15;19(16):4298-309. [Content Brief]

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