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Vhl von Hippel-Lindau [ Drosophila melanogaster (fruit fly) ]

Gene ID: 53433, updated on 4-Jan-2025

Summary

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Official Symbol
Vhlprovided by FlyBase
Official Full Name
von Hippel-Lindauprovided by FlyBase
Primary source
FLYBASE:FBgn0041174
Locus tag
Dmel_CG13221
See related
AllianceGenome:FB:FBgn0041174
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Drosophila melanogaster
Lineage
Eukaryota; Metazoa; Ecdysozoa; Arthropoda; Hexapoda; Insecta; Pterygota; Neoptera; Endopterygota; Diptera; Brachycera; Muscomorpha; Ephydroidea; Drosophilidae; Drosophila; Sophophora
Also known as
BcDNA:RH61560; CG13221; d-vhl; d-VHL; Dmel\CG13221; dmVHL; dVHL; Dvhl; DVhl; vhl; VHL
Summary
Involved in several processes, including microtubule cytoskeleton organization; protein export from nucleus; and respiratory system development. Part of VCB complex. Is expressed in embryonic/larval tracheal dorsal trunk; embryonic/larval tracheal transverse connective; embryonic/larval visceral tracheal branch; and organism. Used to study 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 several human genes including VHL (von Hippel-Lindau tumor suppressor). [provided by Alliance of Genome Resources, Jan 2025]
Orthologs
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Genomic context

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See Vhl in Genome Data Viewer
Location:
47E5-47E6; 2-64 cM
Exon count:
1
Annotation release Status Assembly Chr Location
Release 6.54 current Release 6 plus ISO1 MT (GCF_000001215.4) 2R NT_033778.4 (11279725..11281033)
Release 5.57 previous assembly Release 5 (GCF_000001215.2) 2R NT_033778.3 (7167230..7168538)

Chromosome 2R - NT_033778.4Genomic Context describing neighboring genes Neighboring gene Cuticular protein 47Eg Neighboring gene uncharacterized protein Neighboring gene uncharacterized protein Neighboring gene uncharacterized protein Neighboring gene F-box and leucine-rich repeat protein 6 Neighboring gene uncharacterized protein

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Bibliography

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Related articles in PubMed

  1. Arginine refolds, stabilizes, and restores function of mutant pVHL proteins in animal model of the VHL cancer syndrome. Shmueli MD, et al. Oncogene, 2019 Feb. PMID 30194449
  2. Computational and experimental characterization of dVHL establish a Drosophila model of VHL syndrome. Shmueli MD, et al. PLoS One, 2014. PMID 25310726, Free PMC Article
  3. Analysis of the hypoxia-sensing pathway in Drosophila melanogaster. Arquier N, et al. Biochem J, 2006 Jan 15. PMID 16176182, Free PMC Article
  4. Comparative Expression Profiling of Wild Type Drosophila Malpighian Tubules and von Hippel-Lindau Haploinsufficient Mutant. Ignesti M, et al. Front Physiol, 2019. PMID 31191337, Free PMC Article
  5. pVHL-mediated SMAD3 degradation suppresses TGF-β signaling. Zhou J, et al. J Cell Biol, 2022 Jan 3. PMID 34860252, Free PMC Article

See all (58) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Von Hippel-Lindau tumor suppressor (VHL) stimulates TOR signaling by interacting with phosphoinositide 3-kinase (PI3K).
    Title: Von Hippel-Lindau tumor suppressor (VHL) stimulates TOR signaling by interacting with phosphoinositide 3-kinase (PI3K).
  2. In both models, L- as well as D-arginine enhanced the ability of wild-type pVHL and certain misfolded mutant versions of pVHL to bind ODD, the HIF-derived target peptide, reflecting restoration of pVHL function. Moreover, continuous feeding of Drosophila expressing misfolded versions of pVHL either L- or D-arginine rich diet rescued their lethal phenotype.
    Title: Arginine refolds, stabilizes, and restores function of mutant pVHL proteins in animal model of the VHL cancer syndrome.
  3. Interaction between Nm23 and the tumor suppressor VHL
    Title: Interaction between Nm23 and the tumor suppressor VHL.
  4. Transgenic pVHL can fully maintain normal dVHL-HIF1-alpha downstream pathways in flies.
    Title: Computational and experimental characterization of dVHL establish a Drosophila model of VHL syndrome.
  5. Codepletion of Vhl with Mgr gives partial rescue of tubulin instability, monopolar spindle formation, and loss of centrosomes.
    Title: Drosophila Mgr, a Prefoldin subunit cooperating with von Hippel Lindau to regulate tubulin stability.
  6. The results establish a developmental function of the VHL gene that is relevant to its tumor-suppressor activity.
    Title: Drosophila VHL tumor-suppressor gene regulates epithelial morphogenesis by promoting microtubule and aPKC stability.
  7. findings reveal a second type of tracheal hypoxic response in which Sima activation conflicts with developmental tracheogenesis, and identify the dVHL and ago ubiquitin ligases as key determinants of hypoxia sensitivity in tracheal cells
    Title: Regulation of Drosophila embryonic tracheogenesis by dVHL and hypoxia.
Submit: New GeneRIF Correction

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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Homology

Gene Ontology Provided by FlyBase

Function Evidence Code Pubs
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
Process Evidence Code Pubs
involved_in centrosome cycle IGI
Inferred from Genetic Interaction
more info
 PubMed 
involved_in epithelial cell migration, open tracheal system IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in establishment or maintenance of polarity of follicular epithelium IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in lumen formation, open tracheal system IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in mitotic spindle assembly IGI
Inferred from Genetic Interaction
more info
 PubMed 
involved_in negative regulation of microtubule depolymerization IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in open tracheal system development IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in protein export from nucleus IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in protein ubiquitination IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in protein ubiquitination IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in regulation of cellular response to hypoxia IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in trachea development IMP
Inferred from Mutant Phenotype
more info
 PubMed 
Component Evidence Code Pubs
part_of VCB complex IBA
Inferred from Biological aspect of Ancestor
more info
  
part_of VCB complex IDA
Inferred from Direct Assay
more info
 PubMed 
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
Names
CG13221-PA
CG13221-PB
Vhl-PA
Vhl-PB
von hippel lindau
von hippel lindau protein

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

  1. NT_033778.4 Reference assembly

    Range
    11279725..11281033
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_080247.3 → NP_524986.1  von Hippel-Lindau, isoform A [Drosophila melanogaster]

    See identical proteins and their annotated locations for NP_524986.1

    Status: REVIEWED

    UniProtKB/Swiss-Prot
    Q9V3C1
    UniProtKB/TrEMBL
    A0A0B4KEJ4, Q2XYJ4
    Related
    FBpp0087216
    Conserved Domains (1) summary
    cd05468
    Location:21 → 162
    pVHL; von Hippel-Landau (pVHL) tumor suppressor protein

  2. NM_001273956.1 → NP_001260885.1  von Hippel-Lindau, isoform B [Drosophila melanogaster]

    See identical proteins and their annotated locations for NP_001260885.1

    Status: REVIEWED

    UniProtKB/Swiss-Prot
    Q9V3C1
    UniProtKB/TrEMBL
    A0A0B4KEJ4, Q2XYJ4
    Conserved Domains (1) summary
    cd05468
    Location:21 → 162
    pVHL; von Hippel-Landau (pVHL) tumor suppressor protein
Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
A0A0B4KEJ4 A0A0B4KEJ4
Q2XYJ4 Q2XYJ4
Q9V3C1.1 Q9V3C1

Gene LinkOut

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