Marked reduction of Na+, K+-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice

Article ID	Journal	Published Year	Pages	File Type
8344029	Molecular Genetics and Metabolism	2012	6 Pages	PDF

Abstract

âº A knockout model of glutaric aciduria type I (Gcdhâ/â) was supplemented with lysine. âº Creatine kinase activity was inhibited in Gcdhâ/â mice after lysine supplementation. âº Na+, K+- ATPase activity was inhibited in Gcdhâ/â mice after lysine supplementation. âº Acute lysine administration disturbed the cellular bioenergetics in Gcdhâ/â mice.

Keywords

N-[2-hydroxyethyl]piperazine-N′-[2-ethane-sulfonic acid]HEPES α-KGDH TCA Creatine kinase activity Na+, K+-ATPase activity GCDH SPSS DCIP SAL α-ketoglutarate dehydrogenase EDTA 3-hydroxyglutaric acid ethylenediaminetetracetic acid tricarboxylic acid glutaric acid Statistical Package for the Social Sciences dichloroindophenol Bioenergetics Saline Lysine LYS Brain knockout Glutaric acidemia type I wild type Creatine kinase glutaryl-CoA dehydrogenase

Related Topics

Life Sciences Biochemistry, Genetics and Molecular Biology Biochemistry

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Marked reduction of Na+, K+-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice

Authors

Alexandre Umpierrez Amaral, Cristiane Cecatto, Bianca Seminotti, Ãngela Zanatta, Carolina Gonçalves Fernandes, Estela Natacha Brandt Busanello, Luisa Macedo Braga, César Augusto JoÃ£o Ribeiro, Diogo Onofre Gomes de Souza, Michael Woontner,