Protein Information

Cytoscape View


UniProt AC / UniProt ID P40337 / VHL_HUMAN
Protein Name von Hippel-Lindau disease tumor suppressor
Gene Name Name: VHL
Organism Homo sapiens (Human)

PRO ID PR:P40337
DAG View
PRO Name von Hippel-Lindau disease tumor suppressor (human)
Definition A von Hippel-Lindau disease tumor suppressor that is encoded in the genome of human.
Short Label hVHL
Category organism-gene

Interactive Sequence View

Select/align proteoforms across species


P40337 (VHL) as Substrate



Site PTM Type PTM Enzyme Score Source PMID
S33 Phosphorylation P68400 (CSNK2A1) PhosphoSitePlus   RLIMS-P 20637892
S38 Phosphorylation P68400 (CSNK2A1) PhosphoSitePlus   RLIMS-P 20637892
S43 Phosphorylation P68400 (CSNK2A1) PhosphoSitePlus   RLIMS-P 20637892
S68 Phosphorylation P49841 (GSK3B) PhosphoSitePlus 16847331
S72 Phosphorylation P48729 (CSNK1A1) , O14965 (AURKA) PhosphoSitePlus   RLIMS-P 16847331, 30943211, 23785518
R79 Methylation PhosphoSitePlus
S80 Phosphorylation PhosphoSitePlus
T100 Phosphorylation Q96PY6 (NEK1) PhosphoSitePlus 23255108
T105 Phosphorylation Q96PY6 (NEK1) PhosphoSitePlus 23255108
S111 Phosphorylation O96017 (CHEK2) PhosphoSitePlus   Signor 22071692
Y112 Phosphorylation Q96PY6 (NEK1) PhosphoSitePlus 23255108
K159 Ubiquitination PhosphoSitePlus
S168 Phosphorylation Q96PY6 (NEK1) PhosphoSitePlus   RLIMS-P 23255108
K171 Sumoylation PhosphoSitePlus
K171 Ubiquitination PhosphoSitePlus
Y175 Phosphorylation Q96PY6 (NEK1) PhosphoSitePlus 23255108
S183 Phosphorylation Q96PY6 (NEK1) PhosphoSitePlus 23255108
Y185 Phosphorylation PhosphoSitePlus   RLIMS-P 21212839
K196 Ubiquitination PhosphoSitePlus
T202 Phosphorylation Q96PY6 (NEK1) PhosphoSitePlus 23255108


PTM-dependent PPI

PTM type Substrate Site Interactant Association type Source PMID
Phosphorylation P07737 (PFN1) Y129 P40337 (VHL) unknown eFIP 24747440


Proteoform PPIs

Protein 1 Relation Protein 2 Source PMID
PR:P40337-3 (hVHL/iso:3) Interaction PR:O75912 (hDGKI) PRO 17986458


PTM sites affected in variants

Site Variant Source PMID Disease [Sample source]
S33 * Biomuta DOID:263 / kidney cancer [ cosmic ]
S33 A33 Biomuta DOID:263 / kidney cancer [ cosmic ]
S38 F38 Biomuta DOID:263 / kidney cancer [ cosmic ]
S43 F43 Biomuta DOID:263 / kidney cancer [ cosmic ] DOID:11054 / urinary bladder cancer [ cosmic ]
S68 L68 Biomuta DOID:263 / kidney cancer [ cosmic ]
S68 T68 Biomuta DOID:263 / kidney cancer [ cosmic ]
S68 P68 Biomuta DOID:263 / kidney cancer [ cosmic ]
S68 * Biomuta DOID:263 / kidney cancer [ cosmic ]
S72 P72 Biomuta DOID:263 / kidney cancer [ cosmic ]
R79 P79 Biomuta DOID:263 / kidney cancer [ cosmic ]
R79 G79 Biomuta DOID:1324 / lung cancer [ cosmic, icgc ] DOID:11054 / urinary bladder cancer [ cosmic ]
R79 C79 Biomuta DOID:10283 / prostate cancer [ cosmic ] DOID:11054 / urinary bladder cancer [ cosmic ]
R79 H79 Biomuta DOID:1781 / thyroid gland cancer [ cosmic ] DOID:263 / kidney cancer [ cosmic ] DOID:1781 / thyroid cancer [ icgc ]
S80 N80 Biomuta DOID:263 / kidney cancer [ cosmic ]
S80 G80 Biomuta DOID:3953 / adrenal gland cancer [ cosmic ]
S80 I80 Biomuta DOID:263 / kidney cancer [ cosmic ]
S80 R80 Biomuta DOID:1793 / pancreatic cancer [ cosmic ] DOID:263 / kidney cancer [ cosmic, icgc ]
T100 A100 Biomuta DOID:1319 / brain cancer [ cosmic ] DOID:263 / kidney cancer [ cosmic ]
T105 P105 Biomuta DOID:263 / kidney cancer [ cosmic ]
T105 R105 Biomuta DOID:263 / kidney cancer [ cosmic ]
T105 K105 Biomuta DOID:10283 / prostate cancer [ cosmic ]
T105 M105 Biomuta DOID:9256 / colorectal cancer [ cosmic ] DOID:263 / kidney cancer [ cosmic ]
S111 G111 Biomuta DOID:263 / kidney cancer [ cosmic ]
S111 I111 Biomuta DOID:263 / kidney cancer [ cosmic ]
S111 N111 Biomuta DOID:263 / kidney cancer [ cosmic, icgc ]
S111 R111 Biomuta DOID:263 / kidney cancer [ cosmic ]
Y112 * Biomuta DOID:1793 / pancreatic cancer [ cosmic ] DOID:263 / kidney cancer [ cosmic ]
Y112 C112 Biomuta DOID:1793 / pancreatic cancer [ cosmic ] DOID:263 / kidney cancer [ cosmic ]
Y112 D112 Biomuta DOID:263 / kidney cancer [ cosmic ]
K159 E159 Biomuta DOID:3953 / adrenal gland cancer [ cosmic ]
K159 N159 Biomuta DOID:263 / kidney cancer [ cosmic ]
K159 * Biomuta [ cosmic ] DOID:263 / kidney cancer [ cosmic ]
S168 C168 Biomuta DOID:263 / kidney cancer [ cosmic ]
K171 N171 Biomuta DOID:263 / kidney cancer [ cosmic ]
K171 * Biomuta DOID:263 / kidney cancer [ cosmic ]
Y175 * Biomuta DOID:263 / kidney cancer [ cosmic ]
Y175 D175 Biomuta DOID:263 / kidney cancer [ cosmic ]
S183 * Biomuta DOID:263 / kidney cancer [ cosmic ]
S183 L183 Biomuta DOID:263 / kidney cancer [ cosmic ]
Y185 * Biomuta DOID:263 / kidney cancer [ cosmic ]
Y185 F185 Biomuta DOID:9256 / colorectal cancer [ cosmic ]
K196 * Biomuta DOID:263 / kidney cancer [ cosmic ]