NDLI: 3-Methylcrotonylglycine Disrupts Mitochondrial Energy Homeostasis and Inhibits Synaptic Na+,K+-ATPase Activity in Brain of Young Rats

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3-Methylcrotonylglycine Disrupts Mitochondrial Energy Homeostasis and Inhibits Synaptic Na+,K+-ATPase Activity in Brain of Young Rats

Content Provider	Springer Nature Link
Author	Moura, Alana Pimentel Ribeiro, César Augusto João Zanatta, Ângela Busanello, Estela Natacha Brandt Tonin, Anelise Miotti Wajner, Moacir
Copyright Year	2011
Abstract	Deficiency of 3-methylcrotonyl-CoA carboxylase activity is an inherited metabolic disease biochemically characterized by accumulation and high urinary excretion of 3-methylcrotonylglycine (3MCG), and also of 3-hydroisovalerate in lesser amounts. Affected patients usually have neurologic dysfunction, brain abnormalities and cardiomyopathy, whose pathogenesis is still unknown. The present study investigated the in vitro effects of 3MCG on important parameters of energy metabolism, including CO2 production from labeled acetate, enzyme activities of the citric acid cycle, as well as of the respiratory chain complexes I–IV (oxidative phosphorylation), creatine kinase (intracellular ATP transfer), and synaptic Na+,K+-ATPase (neurotransmission) in brain cortex of young rats. 3MCG significantly reduced CO2 production, implying that this compound compromises citric acid cycle activity. Furthermore, 3MCG diminished the activities of complex II-III of the respiratory chain, mitochondrial creatine kinase and synaptic membrane Na+,K+-ATPase. Furthermore, antioxidants were able to attenuate or fully prevent the inhibitory effect of 3MCG on creatine kinase and synaptic membrane Na+,K+-ATPase activities. We also observed that lipid peroxidation was elicited by 3MCG, suggesting the involvement of free radicals on 3MCG-induced effects. Considering the importance of the citric acid cycle and the electron flow through the respiratory chain for brain energy production, creatine kinase for intracellular energy transfer, and Na+,K+-ATPase for the maintenance of the cell membrane potential, the present data indicate that 3MCG potentially impairs mitochondrial brain energy homeostasis and neurotransmission. It is presumed that these pathomechanisms may be involved in the neurological damage found in patients affected by 3-methylcrotonyl-CoA carboxylase deficiency.
Starting Page	297
Ending Page	307
Page Count	11
File Format	PDF
ISSN	02724340
Journal	Cellular and Molecular Neurobiology
Volume Number	32
Issue Number	2
e-ISSN	15736830
Language	English
Publisher	Springer US
Publisher Date	2011-10-13
Publisher Place	Boston
Access Restriction	Subscribed
Subject Keyword	3-Methylcrotonyl-CoA carboxylase deficiency 3-Methylcrotonylglycine Brain bioenergetics Reactive species Cell Biology Neurosciences Neurobiology
Content Type	Text
Resource Type	Article
Subject	Cell Biology Medicine Cellular and Molecular Neuroscience

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