vhl-1 von Hippel-Lindau tumor suppressor homolog [Caenorhabditis elegans] - Gene

Summary

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Official Symbol: vhl-1
Official Full Name: von Hippel-Lindau tumor suppressor homolog
Primary source: WormBase:WBGene00006922
Locus tag: CELE_F08G12.4
See related: AllianceGenome:WB:WBGene00006922
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Caenorhabditis elegans (strain: Bristol N2)
Lineage: Eukaryota; Metazoa; Ecdysozoa; Nematoda; Chromadorea; Rhabditida; Rhabditina; Rhabditomorpha; Rhabditoidea; Rhabditidae; Peloderinae; Caenorhabditis
Summary: Involved in response to hypoxia. Part of Cul2-RING ubiquitin ligase complex. Is expressed widely. Used to study clear cell renal cell carcinoma and von Hippel-Lindau disease. Human ortholog(s) of this gene implicated in several diseases, including pancreatic cancer (multiple); pheochromocytoma; polycythemia (multiple); renal cell carcinoma; and von Hippel-Lindau disease. Orthologous to human VHL (von Hippel-Lindau tumor suppressor). [provided by Alliance of Genome Resources, Dec 2024]
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Genomic context

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See vhl-1 in Genome Data Viewer

Location:: chromosome: X

Exon count:: 3

Sequence:: Chromosome: X; NC_003284.9 (11307356..11308518)

Chromosome X - NC_003284.9 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_003284.9

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Inhibiting von Hippel-Lindau protein-mediated Dishevelled ubiquitination protects against experimental parkinsonism.
Title: Inhibiting von Hippel‒Lindau protein-mediated Dishevelled ubiquitination protects against experimental parkinsonism.
Hypoxic response regulators RHY-1 and EGL-9/PHD promote longevity through a VHL-1-independent transcriptional response.
Title: Hypoxic response regulators RHY-1 and EGL-9/PHD promote longevity through a VHL-1-independent transcriptional response.
The authors identified vhl-1 as a genetic modifier and mitochondria-targeting antioxidants as rescuing pharmacological agents for this previously undescribed dopamine neuron subtype-specific neurodegeneration in C. elegans.
Title: VHL-1 inactivation and mitochondrial antioxidants rescue C. elegans dopaminergic neurodegeneration.
ELC-2 and the von Hippel-Lindau protein VHL-1, components of an Elongin-Cullin-SOCS box (ECS) E3 ubiquitin ligase, modulate feeding behavior after experiencing stress.
Title: An Elongin-Cullin-SOCS Box Complex Regulates Stress-Induced Serotonergic Neuromodulation.
loss of VHL-1 increased life span; deletion of HIF-1 was epistatic to VHL-1, indicating HIF-1 acts downstream of VHL-1 to modulate aging; VHL-1 & HIF-1 control longevity by a mechanism distinct from dietary restriction & insulin-like signaling
Title: Proteasomal regulation of the hypoxic response modulates aging in C. elegans.
there is a discrete HIF-1-independent function of VHL-1 that is connected with extracellular matrix function
Title: Genetic analysis of pathways regulated by the von Hippel-Lindau tumor suppressor in Caenorhabditis elegans.
The Caenorhabditis elegans rhy-1 gene inhibits hif-1 activity in a negative feedback loop that does not include vhl-1.
Title: The Caenorhabditis elegans rhy-1 gene inhibits HIF-1 hypoxia-inducible factor activity in a negative feedback loop that does not include vhl-1.

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Pathways from PubChem

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Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

F08G12.4 (e-PCR)
- UniSTS:318067

Homology

Orthologs from OrthoDB

Gene Ontology Provided by WormBase

Process	Evidence Code	Pubs
involved_in protein ubiquitination	IBA Inferred from Biological aspect of Ancestor more info
involved_in protein ubiquitination	IEA Inferred from Electronic Annotation more info
involved_in response to hypoxia	IMP Inferred from Mutant Phenotype more info	PubMed

Component	Evidence Code	Pubs
part_of Cul2-RING ubiquitin ligase complex	IPI Inferred from Physical Interaction more info	PubMed
part_of VCB complex	IBA Inferred from Biological aspect of Ancestor more info
is_active_in nucleus	IBA Inferred from Biological aspect of Ancestor more info

General protein information

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Preferred Names: von Hippel-Lindau tumor suppressor homolog

NP_509889.1

Confirmed by transcript evidence

NCBI Reference Sequences (RefSeq)

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Genome Annotation

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference assembly

Genomic

NC_003284.9 Reference assembly

Range

11307356..11308518

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_077488.6 → NP_509889.1 von Hippel-Lindau tumor suppressor homolog [Caenorhabditis elegans]

See identical proteins and their annotated locations for NP_509889.1

Status: REVIEWED

UniProtKB/Swiss-Prot

Q19213

Conserved Domains (1) summary

cd05468
Location:19 → 157

pVHL; von Hippel-Landau (pVHL) tumor suppressor protein