Ethylmalonic acid modulates Na+, K+-ATPase activity and mRNA levels in rat cerebral cortex

Schuck, Patricia Fernanda, De Assis, Denis Reis, Viegas, Carolina Maso, Brandao Pereira, Talita Carneiro, Machado, Jessica Luca, Furlanetto, Camila Brulezi, Bogo, Mauricio Reis, Streck, Emilio Luiz and Ferreira, Gustavo Costa (2013) Ethylmalonic acid modulates Na+, K+-ATPase activity and mRNA levels in rat cerebral cortex. Synapse, 67 3: 111-117. doi:10.1002/syn.21618

Author Schuck, Patricia Fernanda
De Assis, Denis Reis
Viegas, Carolina Maso
Brandao Pereira, Talita Carneiro
Machado, Jessica Luca
Furlanetto, Camila Brulezi
Bogo, Mauricio Reis
Streck, Emilio Luiz
Ferreira, Gustavo Costa
Title Ethylmalonic acid modulates Na+, K+-ATPase activity and mRNA levels in rat cerebral cortex
Journal name Synapse   Check publisher's open access policy
ISSN 0887-4476
Publication date 2013-03
Year available 2012
Sub-type Article (original research)
DOI 10.1002/syn.21618
Volume 67
Issue 3
Start page 111
End page 117
Total pages 7
Place of publication Hoboken, NJ, United States
Publisher John Wiley & Sons
Collection year 2013
Language eng
Formatted abstract
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) activity and mRNA levels in cerebral cortex from 30-day-old rats. For in vitro studies, cerebral cortex homogenates were incubated in the presence of EMA at 0.5, 1, or 2.5 mM concentrations for 1 h. For in vivo experiments, animals received three subcutaneous EMA injections (6 μmol g-1; 90-min interval) and were killed 60 min after the last injection. After that, NAK activity and its mRNA expression were measured. We observed that EMA did not affect this enzyme activity in vitro. In contrast, EMA administration significantly increased NAK activity and decreased mRNA NAK expression as assessed by semiquantitative reverse transcriptase polymerase chain reaction when compared with control group. Considering the high score of residues prone to phosphorylation on NAK, this profile can be associated with a possible regulation by specific phosphorylation sites of the enzyme. Altogether, the present results suggest that NAK alterations may be involved in the pathophysiology of brain damage found in patients in which EMA accumulates.
Keyword Ethylmalonic acid
K+ -ATPase
Short-chain acyl-CoA dehydrogenase deficiency
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ
Additional Notes Article first published online: 6 December 2012.

Document type: Journal Article
Sub-type: Article (original research)
Collections: Queensland Brain Institute Publications
Official 2013 Collection
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