Oxidative stress contributes to outcome severity in a Drosophila melanogaster model of classic galactosemia.

ABSTRACT: Classic galactosemia is a genetic disorder that results from profound loss of galactose-1P-uridylyltransferase (GALT). Affected infants experience a rapid escalation of potentially lethal acute symptoms following exposure to milk. Dietary restriction of galactose prevents or resolves the acute sequelae; however, many patients experience profound long-term complications. Despite decades of research, the mechanisms that underlie pathophysiology in classic galactosemia remain unclear. Recently, we developed a Drosophila melanogaster model of classic galactosemia and demonstrated that, like patients, GALT-null Drosophila succumb in development if exposed to galactose but live if maintained on a galactose-restricted diet. Prior models of experimental galactosemia have implicated a possible asso

SUBMITTER: Jumbo-Lucioni PP 

PROVIDER: S-EPMC3529341 | biostudies-literature | 2013 Jan

REPOSITORIES: biostudies-literature