Von Hippel-Lindau protein

Ohh, M., et al., Ubiquitination of hypoxia-indudble factor requires direct binding to the -domain of the von Hippel-Lindau protein. Nat Cell Biol, 2000, 2(7), 423-7. 

Coen, P. G., McDonald, E. R., 3rd, Herman, J. G., and El-Deiey, W. S. Tat-binding protein-1, a component of the 26 S proteasome, contributes to the E3 ubiquitin ligase function of the von Hippel-Lindau protein. Nat Genet... 

Sang N, Fang J, Srinivas V, Leshchinsky I, Caro J. Carboxyl-terminal transactivation activity of hypoxia-inducible factor 1 alpha is governed by a von Hippel-Lindau protein-independent, hydroxylation-regulated association with p300/CBP. Mol. Cell. Biol. 2002 22 2984-2992. 

Kaelin WG Jr. The von Hippel-Lindau protein, HIP hydroxylation, and oxygen sensing. Biochem. Biophys. Res. Commun. 142. 2005 338 627-638. 

ER. Neutrophils from patients with heterozygous germline mutations in the von Hippel Lindau protein (pVHL) display delayed apoptosis and enhanced bacterial phagocytosis. Blood 2006 108 3176-3178. 

Figure 22. Schematic of proteosome dependent degradation of HIF-la. Normoxia enhances HIF-la degradation and hypoxia prevents it. Hydroxylation by oxygen sensing enzymes (PHD2) and ubiquitination by a E3-ubiquitin ligase complex containing the von Hippel Lindau protein (pVHL) are critical steps of HIF-la degradation process. See text for a more detailed explanation.

Iliopoulos O, Levy AP, Jiang C, et al. Negative regulation of hypoxia-inducible genes by the von Hippel-Lindau protein. Proc Natl Acad Sci USA. 1996 93 10595. 

Los M, Jansen GH, Kaebn WG, Lips CJM, Blijham GH, Voest EE. Expression pattern of the von Hippel-Lindau protein in human tissues. Lab Invest 1996 75 231-238. 

Genbacev O, Krtolica A, Kaelin W, Fisher SJ. Human C3fiotrophoblast expression of the von Hippel-Lindau protein is downregulated during uterine invasion in situ and upregulated by hypoxia in vitro. Dev Biol 2001 233 526-536. 

Pioli PA, Rigby WFC. The von Hippel-Lindau protein interacts with hnRNP A2 and regulates its expression. J Biol Chem 2001 276 40346-40352. 

Hoffinan MA, Ohh M, Yang H, Klco JM, Ivan M, KaeUn WGJ. von Hippel-Lindau protein mutants linked to type 2C VHL disease preserve the ability to downregulate HIF. Hum Mol Genet 2001 10 1019-1027. 

Kroll SL, Paulding WR, SchneU PO, Barton MC, Conaway JW, Conaway RC, Czyzyk-Krzeska MR Von Hippel-Lindau protein induces hypoxia-regulated arrest of tyrosine hydroxylase transcript elongation in pheochromocytoma cells. J Biol Chem 1999 274 30109-30114. 

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