Annotation Detail

Information
Associated Genes
ARSA
Associated Variants
ARSA p.Ile181Ser (p.I181S) ( ENST00000216124.10, ENST00000356098.9, ENST00000395619.3, ENST00000395621.7, ENST00000453344.6 )
ARSA p.Ile181Ser (p.I181S) ( ENST00000216124.10, ENST00000356098.9, ENST00000395619.3, ENST00000395621.7, ENST00000453344.6 )
Associated Disease
Leukodystrophy, Metachromatic
Source Database
DisGeNET
Description
It seems that I179S mutation on one allele with another mutation on the other allele reduces ASA activity, but the enzyme can still cope with a part of the substrate influx, leading to late-juvenile-onset MLD with such strikingly similar phenotypes remaining a little bit of the adult (psychiatric) type.
Pubmed
9007312
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.514051056554323
Year of publication
1996
Drugs