Annotation Detail

Information
Associated Genes
VHL
Associated Variants
VHL p.Gln164Arg (p.Q164R) ( ENST00000256474.3, ENST00000345392.3, ENST00000696143.2, ENST00000696153.1, ENST00000713811.1, ENST00000713812.1, ENST00000713815.1, ENST00000713982.1 )
VHL p.Gln164Arg (p.Q164R) ( ENST00000256474.3, ENST00000345392.3, ENST00000696143.2, ENST00000696153.1, ENST00000713811.1, ENST00000713812.1, ENST00000713815.1, ENST00000713982.1 )
Associated Disease
pheochromocytoma
Source Database
DisGeNET
Description
The same VHL mutation has been reported in a patient who developed a pheochromocytoma at the age of 10 years; therefore, for known VHL Q164R mutation carriers, we suggest screening for pheochromocytoma beginning at 2 years of age.
Pubmed
20583150
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.415849807388327
Year of publication
2010
Drugs