Annotation Detail

Information
Associated Genes
KCNJ11
Associated Variants
KCNJ11 p.Arg201Leu (p.R201L) ( ENST00000528731.1, ENST00000339994.5, ENST00000682350.1, ENST00000682764.1 )
KCNJ11 p.Arg201His (p.R201H) ( ENST00000682764.1, ENST00000682350.1, ENST00000339994.5, ENST00000528731.1 )
KCNJ11 p.Val59Gly (p.V59G) ( ENST00000528731.1, ENST00000682350.1, ENST00000682764.1, ENST00000339994.5 )
KCNJ11 p.Val59Met (p.V59M) ( ENST00000339994.5, ENST00000682350.1, ENST00000682764.1, ENST00000528731.1 )
KCNJ11 p.Arg201Leu (p.R201L) ( ENST00000339994.5, ENST00000528731.1, ENST00000682350.1, ENST00000682764.1 )
KCNJ11 p.Arg201His (p.R201H) ( ENST00000339994.5, ENST00000528731.1, ENST00000682350.1, ENST00000682764.1 )
KCNJ11 p.Val59Gly (p.V59G) ( ENST00000339994.5, ENST00000528731.1, ENST00000682350.1, ENST00000682764.1 )
KCNJ11 p.Val59Met (p.V59M) ( ENST00000339994.5, ENST00000528731.1, ENST00000682350.1, ENST00000682764.1 )
Associated Disease
epilepsy
Source Database
DisGeNET
Description
Recent studies have shown that heterozygous mutations in KCNJ11, which encodes Kir6.2, the pore-forming subunit of the ATP-sensitive potassium (K(ATP)) channel, cause permanent neonatal diabetes either alone (R201C, R201H) or in association with developmental delay, muscle weakness and epilepsy (V59G,V59M).
Pubmed
16087682
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.0103445857040439
Year of publication
2005
Drugs