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VHL

HGNC ↗

3 article(s) in the watch · Cancer genetics

VHL encodes a protein regulating the cellular hypoxia response (HIF degradation). Its loss of function causes von Hippel-Lindau disease, a multi-tumour predisposition.

InheritanceAutosomal dominant
Clinical spectrumHaemangioblastomas (CNS, retina), clear-cell renal cancer, phaeochromocytoma, pancreatic NETs
ManagementMulti-organ surveillance protocol; belzutifan (HIF-2α inhibitor) in selected manifestations.

Curated publications

6/10

Hereditary kidney tumor syndromes: structured evaluation of a questionnaire-based approach.

Hereditary renal tumor predisposition syndromes (VHL, TSC, BHD, HLRCC)

Onco16 June 2026
6/10

Comparison of clinical characteristics between patients with single mutation and co-mutation in hereditary renal cancer: a retrospective analysis of 115 patients with von Hippel-Lindau syndrome.

Von Hippel-Lindau syndrome with hereditary clear cell renal cell carcinoma

Onco27 May 2026
5/10

Hereditary Pheochromocytoma as a Major Manifestation of von Hippel Lindau Disease (vHL) in Childhood: Long-term Follow-up of Five Patients with vHL from One Family.

Von Hippel-Lindau disease type 2 with pediatric pheochromocytoma

Onco27 May 2026

External references

OMIM ↗GeneReviews ↗ClinVar ↗NCBI Gene ↗
Geno'X · Genetic watch

Weekly review of the literature in constitutional genetics (new genes, mechanisms, syndromes, functional studies). Designed for medical biologists, geneticists, trainees and researchers who want to stay up to date without burning the midnight oil.

The summaries presented are original syntheses. Copyrighted abstracts are not reproduced verbatim — a direct link to the publication is always provided.

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Content for informational purposes — does not replace professional medical advice.