Annotation Detail

Information
Associated Genes
VHL
Associated Variants
VHL p.Glu70Ter (p.E70*) ( ENST00000345392.3, ENST00000256474.3, ENST00000713815.1, ENST00000713982.1, ENST00000696143.2, ENST00000696153.1, ENST00000713811.1, ENST00000713812.1 )
VHL p.Glu70Ter (p.E70*) ( ENST00000256474.3, ENST00000345392.3, ENST00000696143.2, ENST00000696153.1, ENST00000713811.1, ENST00000713812.1, ENST00000713815.1, ENST00000713982.1 )
Associated Disease
von Hippel-Lindau disease
Source Database
CIViC Evidence
Description
In a study of 114 unrelated VHL families, 85 germline mutations were found. VHL mutations were detected in affected family members, but not in unaffected family members or 96 normal individuals. This nonsense variant was found in a VHL type 1 patient with CNS hemangioblastomas (family ID 4479).
Variant Origin
N/A
Variant Origin
N/A
Evidence URL
https://civic.genome.wustl.edu/links/evidence_items/6059
Gene URL
https://civic.genome.wustl.edu/links/genes/58
Variant URL
https://civic.genome.wustl.edu/links/variants/1834
Rating
4
Evidence Type
Predisposing
Disease
Von Hippel-Lindau Disease
Evidence Direction
Supports
Evidence Level
C
Clinical Significance
Uncertain Significance
Pubmed
7728151
Drugs