Articles with "l2hga patients" as a keyword



Oxidative stress among L-2-hydroxyglutaric aciduria disease patients: evaluation of dynamic thiol/disulfide homeostasis

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Published in 2018 at "Metabolic Brain Disease"

DOI: 10.1007/s11011-018-0354-8

Abstract: L-2-hydroxyglutaric aciduria (L2HGA) is an autosomal recessive disorder that is caused by deficiency of 2-hydroxyglutarate dehydrogenase. Pathophysiology of brain damage is poorly understood. In recent years, it was proposed that oxidative stress was elevated and… read more here.

Keywords: homeostasis; stress; hydroxyglutaric aciduria; l2hga patients ... See more keywords