Annotation Detail

Information
Associated Genes
SOX10
Associated Variants
SOX10 p.Gln377Ter (p.Q377*), POLR2F c.293+6597G>A ( ENST00000407936.5, ENST00000360880.6, ENST00000405557.5, ENST00000698177.1, ENST00000396884.8 )
SOX10 p.Glu189Ter (p.E189*), POLR2F c.294-8155C>A ( ENST00000405557.5, ENST00000407936.5, ENST00000360880.6, ENST00000396884.8, ENST00000698177.1 )
SOX10 p.Ser135Thr (p.S135T), POLR2F c.294-2773C>G ( ENST00000405557.5, ENST00000407936.5, ENST00000360880.6, ENST00000396884.8, ENST00000698177.1 )
SOX10 p.Gln377Ter (p.Q377*), POLR2F c.293+6597G>A ( ENST00000405557.5, ENST00000407936.5, ENST00000396884.8, ENST00000698177.1, ENST00000360880.6 )
SOX10 p.Glu189Ter (p.E189*), POLR2F c.294-8155C>A ( ENST00000405557.5, ENST00000407936.5, ENST00000360880.6, ENST00000396884.8, ENST00000698177.1 )
SOX10 p.Ser135Thr (p.S135T), POLR2F c.294-2773C>G ( ENST00000405557.5, ENST00000407936.5, ENST00000360880.6, ENST00000396884.8, ENST00000698177.1 )
Associated Disease
Megacolon, not Hirschsprung's
Source Database
DisGeNET
Description
The SOX10 mutations chosen were E189X, Q377X, and 482ins6, which are associated with WS4, and S135T that is associated with Yemenite deaf-blind hypopigmentation syndrome (YDBS), which does not manifest megacolon.
Pubmed
16921166
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.00081432561624091
Year of publication
2006
Drugs