Annotation Detail

Information
Associated Genes
INS
Associated Variants
INS MUTATION
INS MUTATION
INS p.Arg89Leu (p.R89L), INS-IGF2 c.187+866G>T ( ENST00000512523.1, ENST00000381330.5, ENST00000397270.1, ENST00000397262.5, ENST00000250971.7 )
INS p.Arg89Pro (p.R89P), INS-IGF2 c.187+866G>C ( ENST00000397270.1, ENST00000381330.5, ENST00000397262.5, ENST00000250971.7, ENST00000512523.1 )
INS p.Arg89His (p.R89H), INS-IGF2 c.187+866G>A ( ENST00000397270.1, ENST00000381330.5, ENST00000397262.5, ENST00000250971.7, ENST00000512523.1 )
INS p.Gly84Arg (p.G84R), INS-IGF2 c.187+850G>A ( ENST00000250971.7, ENST00000397262.5, ENST00000381330.5, ENST00000397270.1, ENST00000512523.1 )
INS p.His34Asp (p.H34D), INS-IGF2 p.His34Asp (p.H34D) ( ENST00000381330.5, ENST00000397270.1, ENST00000397262.5, ENST00000250971.7, ENST00000512523.1 )
INS p.Arg89Leu (p.R89L), INS-IGF2 c.187+866G>T ( ENST00000250971.7, ENST00000381330.5, ENST00000397262.5, ENST00000512523.1, ENST00000397270.1 )
INS p.Arg89Pro (p.R89P), INS-IGF2 c.187+866G>C ( ENST00000250971.7, ENST00000381330.5, ENST00000397262.5, ENST00000512523.1, ENST00000397270.1 )
INS p.Arg89His (p.R89H), INS-IGF2 c.187+866G>A ( ENST00000250971.7, ENST00000381330.5, ENST00000397262.5, ENST00000512523.1, ENST00000397270.1 )
INS p.Gly84Arg (p.G84R), INS-IGF2 c.187+850G>A ( ENST00000250971.7, ENST00000381330.5, ENST00000397262.5, ENST00000512523.1, ENST00000397270.1 )
INS p.His34Asp (p.H34D), INS-IGF2 p.His34Asp (p.H34D) ( ENST00000250971.7, ENST00000381330.5, ENST00000397262.5, ENST00000512523.1, ENST00000397270.1 )
Associated Disease
Hyperproinsulinemia
Source Database
DisGeNET
Description
These mutant proinsulin proteins accumulate in the endoplasmic reticulum (ER) and are poorly secreted except for G84R and in contrast to wild-type and hyperproinsulinemia-associated mutant proteins (H34D and R89H) which were sorted to secretory granules and efficiently secreted.
Pubmed
20034470
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.122985860592883
Year of publication
2010
Drugs