VHL Chromosome 3
Von Hippel-Lindau tumor suppressor
Upload your DNA to see your personal genotypes for variants in VHL.
What This Gene Does
This gene encodes a component of a ubiquitination complex. The encoded protein is involved in the ubiquitination and degradation of hypoxia-inducible-factor (HIF), which is a transcription factor that plays a central role in the regulation of gene expression by oxygen. In addition to oxygen-related gene expression, this protein plays a role in many other cellular processes including cilia formation, cytokine signaling, regulation of senescence, and formation of the extracellular matrix. Variants of this gene are associated with von Hippel-Lindau syndrome, pheochromocytoma, erythrocytosis, renal cell carcinoma, and cerebellar hemangioblastoma. [provided by RefSeq, Jun 2022]
Associated Conditions (30)
Von Hippel-Lindau syndrome
Chuvash polycythemia
Hereditary cancer-predisposing syndrome
Pheochromocytoma
Nonpapillary renal cell carcinoma
Lung sarcomatoid carcinoma
VHL-related disorder
See cases
Congenital Erythrocytosis
Ovarian cancer
Inherited phaeochromocytoma and paraganglioma excluding NF1
Acute leukemia of ambiguous lineage
Papillary renal cell carcinoma type 1
Hereditary cancer
Maffucci syndrome
Clear cell carcinoma of kidney
Cerebellar hemangioblastoma
Skin adenoma
RENAL CELL CARCINOMA WITH PARANEOPLASTIC ERYTHROCYTOSIS
Familial prostate cancer
+10 more conditions
Key Variants
RS1003364563
Conflicting classifications of pathogenicity
Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome
Health Risk
RS1004620245
Conflicting classifications of pathogenicity
Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome
Health Risk
RS1014417508
Conflicting classifications of pathogenicity
Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome
Health Risk
RS1017141110
Conflicting classifications of pathogenicity
Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome
Health Risk
RS1034934219
Conflicting classifications of pathogenicity
Pheochromocytoma, Von Hippel-Lindau syndrome, Chuvash polycythemia
Health Risk
RS104893826
Conflicting classifications of pathogenicity
Pheochromocytoma, Hereditary cancer-predisposing syndrome, Von Hippel-Lindau syndrome
Health Risk
RS104893830
Conflicting classifications of pathogenicity
Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome
Health Risk
RS1051892430
Conflicting classifications of pathogenicity
Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome
Health Risk
RS1054796088
Conflicting classifications of pathogenicity
Von Hippel-Lindau syndrome, Chuvash polycythemia, Pheochromocytoma
Health Risk
RS1056226386
Conflicting classifications of pathogenicity
Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome
Health Risk
RS1057519261
Conflicting classifications of pathogenicity
Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome
Health Risk
RS1057522448
Conflicting classifications of pathogenicity
Von Hippel-Lindau syndrome, Von Hippel-Lindau syndrome
Health Risk
All Variants (437)
| RSID | Category | Clinical Significance | Conditions |
|---|---|---|---|
| RS372483939 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, VHL-related disorder |
| RS375401722 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS398123482 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome, Von Hippel-Lindau syndrome |
| RS5030648 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS5030803 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Pheochromocytoma |
| RS5030819 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS5030823 | Health Risk | Conflicting classifications of pathogenicity | Papillary renal cell carcinoma type 1, Von Hippel-Lindau syndrome, Chuvash polycythemia |
| RS532018719 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS549807529 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS552290225 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS561874453 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome, Chuvash polycythemia |
| RS578091032 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS587778744 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS587780730 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS587780731 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS587780732 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS587780992 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Von Hippel-Lindau syndrome |
| RS61758376 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS727503743 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS730882030 | Health Risk | Conflicting classifications of pathogenicity | Hereditary cancer-predisposing syndrome, Chuvash polycythemia, Von Hippel-Lindau syndrome |
| RS730882033 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Von Hippel-Lindau syndrome |
| RS745439844 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Von Hippel-Lindau syndrome |
| RS745901803 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS747861291 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS749091984 | Health Risk | Conflicting classifications of pathogenicity | Hereditary cancer-predisposing syndrome, Von Hippel-Lindau syndrome, Chuvash polycythemia |
| RS749774529 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS752980085 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS757781272 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS760690217 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS764358108 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Von Hippel-Lindau syndrome |
| RS766576246 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS768390987 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Nonpapillary renal cell carcinoma |
| RS768650092 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS769658318 | Health Risk | Conflicting classifications of pathogenicity | Hereditary cancer-predisposing syndrome, Von Hippel-Lindau syndrome, Chuvash polycythemia |
| RS772944298 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome, Chuvash polycythemia |
| RS773556807 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS774380450 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS774557051 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS776399733 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS776768994 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Von Hippel-Lindau syndrome |
| RS777130107 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Pheochromocytoma |
| RS778005138 | Health Risk | Conflicting classifications of pathogenicity | VHL-related disorder, Von Hippel-Lindau syndrome, VHL-related disorder |
| RS778205243 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS786202857 | Health Risk | Conflicting classifications of pathogenicity | Hereditary cancer-predisposing syndrome, Chuvash polycythemia, Von Hippel-Lindau syndrome |
| RS794726890 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Von Hippel-Lindau syndrome |
| RS863224371 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS863224688 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Chuvash polycythemia, Hereditary cancer-predisposing syndrome |
| RS863224839 | Health Risk | Conflicting classifications of pathogenicity | Chuvash polycythemia, Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome |
| RS864622267 | Health Risk | Conflicting classifications of pathogenicity | Hereditary cancer-predisposing syndrome, Chuvash polycythemia, Von Hippel-Lindau syndrome |
| RS864622334 | Health Risk | Conflicting classifications of pathogenicity | Von Hippel-Lindau syndrome, Hereditary cancer-predisposing syndrome, Von Hippel-Lindau syndrome |