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Details on Person Mitochondrial sarcosine dehydrogenase (SARDH) oxidatively de...

Class:IdSummation:6797944
_displayNameMitochondrial sarcosine dehydrogenase (SARDH) oxidatively de...
_timestamp2026-01-14 21:01:17
created[InstanceEdit:6797943] Jassal, Bijay, 2015-09-16
modified[InstanceEdit:6798314] Jassal, Bijay, 2015-09-18
[InstanceEdit:9979091] D'Eustachio, Peter, 2026-01-14
textMitochondrial sarcosine dehydrogenase (SARDH) oxidatively demethylates sarcosine (SARC, aka methylglycine) to glycine (Gly) (Eschenbrenner & Jorns 1999, Wittwer & Wagner 1981). SARDH requires one FAD as cofactor, which is reduced during the reaction. Defects in SARDH cause sarcosinemia (SARCOS; MIM:268900), a disorder characterised by an increased concentration of sarcosine in plasma and increased sarcosine excretion in urine. The clinical phenotypes of sarcosinemia are diverse, ranging from normal (most common) to ones associated with mental retardation, growth delay and muscular abnormalities (Bar-joseph et al. 2012).
(summation)[Reaction:6797913] SARDH:FAD oxidatively demethylates SARC to Gly [Homo sapiens]
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