von Hippel-Lindau (VHL) Protein

R. C. Conaway; J. W. Conaway

doi:10.1016/B978-0-12-378630-2.00333-9

von Hippel-Lindau (VHL) Protein

R. C. Conaway, J. W. Conaway

Research output: Chapter in Book/Report/Conference proceeding › Chapter

2 Scopus citations

Abstract

The von Hippel-Lindau (VHL) protein is a tumor suppressor. Mutations in the VHL protein can give rise to tumors of multiple organ systems, including the central nervous system, the endocrine system, and the kidney. The VHL protein functions as a subunit of a multiprotein ubiquitin ligase that negatively regulates expression of a large collection of hypoxia-inducible genes controlled by hypoxia-inducible transcription factors (HIFs). The VHL ubiquitin ligase prevents inappropriate expression of these hypoxia-inducible genes when cells are grown in a plentiful supply of oxygen by targeting HIFs for rapid ubiquitylation and degradation by the proteasome.

Original language	English (US)
Title of host publication	Encyclopedia of Biological Chemistry
Subtitle of host publication	Second Edition
Publisher	Elsevier Inc.
Pages	570-571
Number of pages	2
ISBN (Electronic)	9780123786319
ISBN (Print)	9780123786302
DOIs	https://doi.org/10.1016/B978-0-12-378630-2.00333-9
State	Published - Feb 15 2013
Externally published	Yes

Keywords

Cullin protein
Dioxygenase
Elongin B
Elongin C
HIF
Hypoxia
Prolyl hydroxylation
Renal carcinoma
Ring finger protein
Transcription
Ubiquitin
VEGF
VHL

ASJC Scopus subject areas

General Biochemistry, Genetics and Molecular Biology

Access to Document

10.1016/B978-0-12-378630-2.00333-9

Cite this

@inbook{efbef14df1fd41b39c095756ebd12bde,

title = "von Hippel-Lindau (VHL) Protein",

abstract = "The von Hippel-Lindau (VHL) protein is a tumor suppressor. Mutations in the VHL protein can give rise to tumors of multiple organ systems, including the central nervous system, the endocrine system, and the kidney. The VHL protein functions as a subunit of a multiprotein ubiquitin ligase that negatively regulates expression of a large collection of hypoxia-inducible genes controlled by hypoxia-inducible transcription factors (HIFs). The VHL ubiquitin ligase prevents inappropriate expression of these hypoxia-inducible genes when cells are grown in a plentiful supply of oxygen by targeting HIFs for rapid ubiquitylation and degradation by the proteasome.",

keywords = "Cullin protein, Dioxygenase, Elongin B, Elongin C, HIF, Hypoxia, Prolyl hydroxylation, Renal carcinoma, Ring finger protein, Transcription, Ubiquitin, VEGF, VHL",

author = "Conaway, {R. C.} and Conaway, {J. W.}",

year = "2013",

month = feb,

day = "15",

doi = "10.1016/B978-0-12-378630-2.00333-9",

language = "English (US)",

isbn = "9780123786302",

pages = "570--571",

booktitle = "Encyclopedia of Biological Chemistry",

publisher = "Elsevier Inc.",

}

TY - CHAP

T1 - von Hippel-Lindau (VHL) Protein

AU - Conaway, R. C.

AU - Conaway, J. W.

PY - 2013/2/15

Y1 - 2013/2/15

N2 - The von Hippel-Lindau (VHL) protein is a tumor suppressor. Mutations in the VHL protein can give rise to tumors of multiple organ systems, including the central nervous system, the endocrine system, and the kidney. The VHL protein functions as a subunit of a multiprotein ubiquitin ligase that negatively regulates expression of a large collection of hypoxia-inducible genes controlled by hypoxia-inducible transcription factors (HIFs). The VHL ubiquitin ligase prevents inappropriate expression of these hypoxia-inducible genes when cells are grown in a plentiful supply of oxygen by targeting HIFs for rapid ubiquitylation and degradation by the proteasome.

AB - The von Hippel-Lindau (VHL) protein is a tumor suppressor. Mutations in the VHL protein can give rise to tumors of multiple organ systems, including the central nervous system, the endocrine system, and the kidney. The VHL protein functions as a subunit of a multiprotein ubiquitin ligase that negatively regulates expression of a large collection of hypoxia-inducible genes controlled by hypoxia-inducible transcription factors (HIFs). The VHL ubiquitin ligase prevents inappropriate expression of these hypoxia-inducible genes when cells are grown in a plentiful supply of oxygen by targeting HIFs for rapid ubiquitylation and degradation by the proteasome.

KW - Cullin protein

KW - Dioxygenase

KW - Elongin B

KW - Elongin C

KW - HIF

KW - Hypoxia

KW - Prolyl hydroxylation

KW - Renal carcinoma

KW - Ring finger protein

KW - Transcription

KW - Ubiquitin

KW - VEGF

KW - VHL

UR - http://www.scopus.com/inward/record.url?scp=85042764407&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85042764407&partnerID=8YFLogxK

U2 - 10.1016/B978-0-12-378630-2.00333-9

DO - 10.1016/B978-0-12-378630-2.00333-9

M3 - Chapter

AN - SCOPUS:85042764407

SN - 9780123786302

SP - 570

EP - 571

BT - Encyclopedia of Biological Chemistry

PB - Elsevier Inc.

ER -

von Hippel-Lindau (VHL) Protein

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this