Annotation Detail

Information
Associated Genes
VHL
Associated Variants
VHL p.Ala149Thr (p.A149T) ( ENST00000256474.3, ENST00000345392.3, ENST00000696143.2, ENST00000696153.1, ENST00000713811.1, ENST00000713812.1, ENST00000713815.1, ENST00000713982.1 )
VHL p.Ala149Thr (p.A149T) ( 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 missense mutation was found in 2 family members with VHL (Family ID 3969 in publication). One carried pheochromocytoma, while both carried angiomas.
Variant Origin
germline
Variant Origin
Rare Germline
Evidence URL
https://civic.genome.wustl.edu/links/evidence_items/4909
Gene URL
https://civic.genome.wustl.edu/links/genes/58
Variant URL
https://civic.genome.wustl.edu/links/variants/1744
Rating
2
Evidence Type
Predisposing
Disease
Von Hippel-Lindau Disease
Evidence Direction
Supports
Evidence Level
C
Clinical Significance
Uncertain Significance
Pubmed
7728151
Drugs