Annotation Detail

Information
Associated Genes
VHL
Associated Variants
VHL MUTATION
VHL MUTATION
Associated Disease
von Hippel-Lindau disease
Source Database
CIViC Evidence
Description
Screening of 92 unrelated patients with VHL disease revealed 61 DNA variants. No variants were found in 96 control cases. This mutation was found in a VHL type 1 patient (patient no. V287). Only moderate evidence of pathogenicity since the protein length changes as a result of an in-frame deletion in a nonrepeat region (AMCG code: PM4).
Variant Origin
germline
Variant Origin
Rare Germline
Evidence URL
https://civic.genome.wustl.edu/links/evidence_items/5653
Gene URL
https://civic.genome.wustl.edu/links/genes/58
Variant URL
https://civic.genome.wustl.edu/links/variants/2099
Rating
2
Evidence Type
Predisposing
Disease
Von Hippel-Lindau Disease
Evidence Direction
Supports
Evidence Level
C
Clinical Significance
Uncertain Significance
Pubmed
9829912
Drugs