Marquise L Hopson

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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(More)
Classic galactosemia (CG) is a potentially lethal inborn error of metabolism that results from the profound loss of galactose-1-phosphate uridylyltransferase (GALT), the second enzyme in the Leloir pathway of galactose metabolism. Neonatal detection and dietary restriction of galactose minimizes or resolves the acute sequelae of CG, but fails to prevent the(More)
AIMS The goal of this study was to use two manganese (Mn)-based superoxide dismutase (SOD) mimics to test the hypothesis that reactive oxygen species contribute to both acute and long-term outcomes in a galactose-1P uridylyltransferase (GALT)-null Drosophila melanogaster model of classic galactosemia. RESULTS We tested the impact of each of two Mn(More)
RATIONALE Cardiolipin (CL), a glycerophospholipid containing four acyl chains, is found in most organisms including Gram-negative bacteria such as Escherichia coli. While CL composes only a fraction of the total glycerophospholipids, the four acyl chains lead to a large number of possible molecular species as defined by the total number of carbons and(More)
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