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Literature DB >> 12716015

Creatine kinase activity from rat brain is inhibited by branched-chain amino acids in vitro.

Carmen Pilla¹, Rui Felipe de Oliveira Cardozo, Carlos Severo Dutra-Filho, Angela Terezinha Souza Wyse, Moacir Wajner, Clóvis Milton Duval Wannmacher.

Abstract

Maple syrup urine disease (MSUD) is an inherited metabolic disorder biochemically characterized by the accumulation of branched-chain amino acids (BCAAs) and their branched-chain keto acids (BCKAs) in blood and other tissues. Neurological dysfunction is usually present in the affected patients, but the mechanisms of brain damage in this disease are not fully understood. Considering that brain energy metabolism seems to be altered in MSUD, the main objective of this study was to investigate the in vitro effect of BCAAs and BCKAs on creatine kinase activity, a key enzyme of energy homeostasis, in brain cortex of young rats. BCAAs, but not their BCKAs, significantly inhibited creatine kinase activity at concentrations similar to those found in the plasma of MSUD patients (0.5-5 mM). Considering the crucial role creatine kinase plays in energy homeostasis in brain, if this effect also occurs in the brain of MSUD patients, it is possible that inhibition of this enzyme activity may contribute to the brain damage found in this disease.

Entities: Chemical Disease Species

Mesh：

Substances：

Year: 2003 PMID： 12716015 DOI： 10.1023/a:1022876130038

Source DB: PubMed Journal: Neurochem Res ISSN： 0364-3190 Impact factor: 3.996

32 in total

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19 in total

1. Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.

Authors: Cláudia Funchal; Patrícia Fernanda Schuck; André Quincozes Dos Santos; Maria Caroline Jacques-Silva; Carmem Gottfried; Regina Pessoa-Pureur; Moacir Wajner
Journal: Cell Mol Neurobiol Date: 2006-02 Impact factor: 5.046

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Journal: Mol Neurobiol Date: 2014-01-05 Impact factor: 5.590

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Authors: Giselli Scaini; Meline O S Morais; Camila B Furlanetto; Luiza W Kist; Talita C B Pereira; Patrícia F Schuck; Gustavo C Ferreira; Matheus A B Pasquali; Daniel P Gelain; José Cláudio F Moreira; Maurício R Bogo; Emilio L Streck
Journal: Neurochem Res Date: 2015-02-14 Impact factor: 3.996

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Authors: Cláudia Funchal; Carmem Gottfried; Lúcia Maria Vieira de Almeida; André Quincozes dos Santos; Moacir Wajner; Regina Pessoa-Pureur
Journal: Cell Mol Neurobiol Date: 2005-08 Impact factor: 5.046

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Journal: Neurochem Res Date: 2013-01-01 Impact factor: 3.996

10. Reduction of glutamate uptake into cerebral cortex of developing rats by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.

Authors: Cláudia Funchal; Aline Meyer Rosa; Moacir Wajner; Susana Wofchuk; Regina Pessoa Pureur
Journal: Neurochem Res Date: 2004-04 Impact factor: 3.996