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® Total Proteins
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Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  InnateDB  |  Ensembl Protein