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Ethylmalonic acid impairs bioenergetics by disturbing succinate and glutamate oxidation and induces mitochondrial permeability transition pore opening in rat cerebellum.
de Moura Alvorcem L, Britto R, Cecatto C, Cristina Roginski A, Rohden F, Nathali Scholl J, Guma FCR, Figueiró F, Umpierrez Amaral A, Zanatta G, Seminotti B, Wajner M, Leipnitz G. de Moura Alvorcem L, et al. J Neurochem. 2021 Jul;158(2):262-281. doi: 10.1111/jnc.15363. Epub 2021 Apr 28. J Neurochem. 2021. PMID: 33837559 Free article.
Tissue accumulation and high urinary excretion of ethylmalonic acid (EMA) are found in ethylmalonic encephalopathy (EE), an inherited disorder associated with cerebral and cerebellar atrophy whose pathogenesis is poorly established. The in vitro and in vivo effects of EMA …
Tissue accumulation and high urinary excretion of ethylmalonic acid (EMA) are found in ethylmalonic encephalopathy (EE), an inherited disord …
Evidence that Thiosulfate Inhibits Creatine Kinase Activity in Rat Striatum via Thiol Group Oxidation.
Grings M, Parmeggiani B, Moura AP, de Moura Alvorcem L, Wyse ATS, Wajner M, Leipnitz G. Grings M, et al. Neurotox Res. 2018 Oct;34(3):693-705. doi: 10.1007/s12640-018-9934-y. Epub 2018 Jul 28. Neurotox Res. 2018. PMID: 30056533
Aiming to better elucidate the pathophysiology of basal ganglia damage in these disorders, we evaluated the in vivo effects of thiosulfate administration on bioenergetics, oxidative stress, and neural damage in rat striatum. ...
Aiming to better elucidate the pathophysiology of basal ganglia damage in these disorders, we evaluated the in vivo effects of thiosu …