UbiNet

Protein Name: Von Hippel-Lindau disease tumor suppressor

UniprotKB/SwissProt ID: VHL_HUMAN (P40337)

Gene Name: VHL

Organism: Homo sapiens (Human).

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

Other Modifications: View all modification sites in dbPTM

Protein Subcellular Localization: Isoform 1: Cytoplasm. Membrane; Peripheral membrane protein. Nucleus. Note=Found predominantly in the cytoplasm and with less amounts nuclear or membrane-associated. Colocalizes with ADRB2 at the cell membrane. Isoform 3: Cytoplasm. Nucleus. Note=Equally

PDB :
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Graphical Visualization of Ubiquitination Sites:

Overview of Protein Ubiquitination Sites with Functional and Structural Information

InterPro ID	Domain
IPR002714

The ubiquitination sites of VHL_HUMAN

No.	Position	Ubiquitinated Peptide	Secondary Structure	Solvent Accessibility	Substrate Motifs	PubMed ID
1	196	DHPNVQ K DLERLT	CCCCHH H HHHHHH	61.06%		2189047

The interacting network mediated by proteins: VHL_HUMAN

Disease:

Disease database	Database Entry	Disease information
KEGG	H00021	Renal cell carcinoma
KEGG	H00236	Congenital polycythemia; Familial erythrocytosis (ECYT)
KEGG	H00559	von Hippel-Lindau syndrome
OMIM	144700	RENAL CELL CARCINOMA, NONPAPILLARY; RCC ;;HYPERNEPHROMA;; ADENOCARCINOMA OF KIDNEY NONPAPILL
OMIM	171300	PHEOCHROMOCYTOMA PHEOCHROMOCYTOMA, SUSCEPTIBILITY TO
OMIM	193300	VON HIPPEL-LINDAU SYNDROME; VHL VON HIPPEL-LINDAU SYNDROME, MODIFIERS OF, INCLUDED
OMIM	263400	ERYTHROCYTOSIS, FAMILIAL, 2 ;;ECYT2;; ERYTHROCYTOSIS, AUTOSOMAL RECESSIVE BENIGN;; POLYCYTHE
OMIM	608537	VHL GENE; VHL
HPRD	01905	Erythrocytosis, familial, 2
HPRD	01905	Hemangioblastoma, sporadic cerebellar
HPRD	01905	Pheochromocytoma
HPRD	01905	Polycythemia, Chuvash type
HPRD	01905	Renal cell carcinoma
HPRD	01905	Renal cell carcinoma with paraneoplastic erythrocytosis
HPRD	01905	Von Hippel-Lindau syndrome

Metabolic pathway:

Kegg map ID	Pathway
map03008	Ribosome biogenesis in eukaryotes

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