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Ethylmalonic acid

purum, ≥97.0% (T)

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≥97.0% (T)


110-114 °C
112-114 °C (lit.)

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M-H Read et al.
Journal of inherited metabolic disease, 28(6), 1149-1150 (2006-01-26)
We report the fourth case of combined D-and L-2-hydroxyglutaric aciduria presenting with neonatal encephalopathy and subependymal cysts.
Patrícia Fernanda Schuck et al.
Synapse (New York, N.Y.), 67(3), 111-117 (2012-11-20)
Ethylmalonic acid (EMA) accumulates in tissues of patients affected by short-chain acyl-CoA dehydrogenase deficiency and ethylmalonic encephalopathy, illnesses characterized by variable neurological symptoms. In this work, we investigated the in vitro and in vivo EMA effects on Na(+), K(+)-ATPase (NAK)...
Patrícia Fernanda Schuck et al.
Neurochemical research, 35(2), 298-305 (2009-09-17)
High concentrations of ethylmalonic acid are found in tissues and biological fluids of patients affected by ethylmalonic encephalopathy, deficiency of short-chain acyl-CoA dehydrogenase activity and other illnesses characterized by developmental delay and neuromuscular symptoms. The pathophysiological mechanisms responsible for the...
Carlo Viscomi et al.
Nature medicine, 16(8), 869-871 (2010-07-27)
Ethylmalonic encephalopathy is caused by mutations in ETHE1, a mitochondrial matrix sulfur dioxygenase, leading to failure to detoxify sulfide, a product of intestinal anaerobes and, in trace amounts, tissues. Metronidazole, a bactericide, or N-acetylcysteine, a precursor of sulfide-buffering glutathione, substantially...
Alethea G Barschak et al.
Metabolic brain disease, 21(1), 11-19 (2006-06-15)
Ethylmalonic aciduria is a common finding in patients affected by short-chain acyl-CoA dehydrogenase (SCAD) deficiency and other diseases characterized by encephalopathy, muscular symptomatology, and lactic acidemia. Considering that the pathophysiological mechanisms of these disorders are practically unknown and that lactic...

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