| VHL 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. Belongs to the VHL family. Expressed in the adult and fetal brain and kidney. 3 alternative splicing and alternative initiation human isoforms have been reported. Note: This description may include information from UniProtKB. |
| Protein type: Tumor suppressor; Ubiquitin ligase |
| Chromosomal Location of human Ortholog: 3p25.3 |
| Cellular Component: cytosol; endoplasmic reticulum; membrane; mitochondrion; nucleoplasm |
| Molecular Function: enzyme binding; protein binding; transcription factor binding; ubiquitin protein ligase activity; ubiquitin-protein transferase activity |
| Biological Process: cell morphogenesis; negative regulation of apoptotic process; negative regulation of cell proliferation; negative regulation of gene expression; negative regulation of JAK-STAT cascade; negative regulation of transcription by RNA polymerase II; negative regulation of transcription from RNA polymerase II promoter in response to hypoxia; positive regulation of cell differentiation; positive regulation of transcription, DNA-templated; post-translational protein modification; protein stabilization; protein ubiquitination; proteolysis; regulation of transcription from RNA polymerase II promoter in response to hypoxia; regulation of transcription, DNA-templated |
| Disease: Erythrocytosis, Familial, 2; Pheochromocytoma; Renal Cell Carcinoma, Nonpapillary; Von Hippel-lindau Syndrome |
| Reference #: P40337 (UniProtKB) |
| Alt. Names/Synonyms: elongin binding protein; HRCA1; Protein G7; pVHL; RCA1; VHL; VHL1; von Hippel-Lindau disease tumor suppressor; von Hippel-Lindau tumor suppressor; von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase |
| Gene Symbols: VHL |
| Molecular weight: 24,153 Da |
| Basal Isoelectric point: 4.7 Predict pI for various phosphorylation states |
| CST Pathways: Angiogenesis |
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology®
|
| Select Structure to View Below |
|---|
VHL |
|
| Cross-references to other databases:
AlphaFold
|
STRING
|
cBioPortal
|
Wikipedia
|
Reactome
|
neXtProt
|
Protein Atlas
|
BioGPS
|
Pfam
|
|