Inhibition of N-acetylglutamate synthase by various monocarboxylic and dicarboxylic short-chain coenzyme A esters and the production of alternative glutamate esters.

Dercksen, M; IJlst, L; Duran, M; Mienie, L J; van Cruchten, A; van der Westhuizen, F H; Wanders, R J A

Dercksen, M; IJlst, L; Duran, M; Mienie, L J; van Cruchten, A; van der Westhuizen, F H; Wanders, R J A.

Afiliação

Dercksen M; Laboratory Genetic Metabolic Diseases, Departments of Pediatrics and Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands; Centre for Human Metabonomics, North-West University (Potchefstroom Campus), Hoffman street 11, Potchefstroom
IJlst L; Laboratory Genetic Metabolic Diseases, Departments of Pediatrics and Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
Duran M; Laboratory Genetic Metabolic Diseases, Departments of Pediatrics and Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
Mienie LJ; Centre for Human Metabonomics, North-West University (Potchefstroom Campus), Hoffman street 11, Potchefstroom, South Africa, 2520.
van Cruchten A; Laboratory Genetic Metabolic Diseases, Departments of Pediatrics and Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
van der Westhuizen FH; Centre for Human Metabonomics, North-West University (Potchefstroom Campus), Hoffman street 11, Potchefstroom, South Africa, 2520.
Wanders RJ; Laboratory Genetic Metabolic Diseases, Departments of Pediatrics and Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.

Biochim Biophys Acta ; 1842(12 Pt A): 2510-6, 2014 Dec.

Article em En | MEDLINE | ID: mdl-23643712

Assuntos

Acil Coenzima A/farmacologia; Aminoácido N-Acetiltransferase/antagonistas & inibidores; Aminoácido N-Acetiltransferase/metabolismo; Ácido Glutâmico/metabolismo; Acil Coenzima A/metabolismo; Ácidos Carboxílicos/metabolismo; Cromatografia Líquida de Alta Pressão/métodos; Ácidos Dicarboxílicos/metabolismo; Relação Dose-Resposta a Droga; Ésteres; Ácido Glutâmico/química; Humanos; Hiperamonemia/metabolismo; Cinética; Espectrometria de Massas em Tandem

Palavras-chave

Acyl-CoAs; Branched-chain amino acids; Hyperammonemia; N-acetylglutamate synthase; N-acylglutamates; Organic acidemia

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Acil Coenzima A / Ácido Glutâmico / Aminoácido N-Acetiltransferase Tipo de estudo: Prognostic_studies Limite: Humans Idioma: En Ano de publicação: 2014 Tipo de documento: Article

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