Structure and biochemical characterization of l-2-hydroxyglutarate dehydrogenase and its role in the pathogenesis of l-2-hydroxyglutaric aciduria.

Yang, Jun; Chen, Xingchen; Jin, Shan; Ding, Jianping

Yang, Jun; Chen, Xingchen; Jin, Shan; Ding, Jianping.

Afiliação

Yang J; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, University of Chinese Academy of Sciences, Shanghai, China.
Chen X; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, University of Chinese Academy of Sciences, Shanghai, China.
Jin S; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, University of Chinese Academy of Sciences, Shanghai, China.
Ding J; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, University of Chinese Academy of Sciences, Shanghai, China; School of Life Science and Technology, ShanghaiTech University, Sh

J Biol Chem ; 300(1): 105491, 2024 Jan.

Article em En | MEDLINE | ID: mdl-37995940

Assuntos

Oxirredutases do Álcool; Encefalopatias Metabólicas Congênitas; Drosophila melanogaster; Modelos Moleculares; Animais; Humanos; Oxirredutases do Álcool/química; Oxirredutases do Álcool/metabolismo; Encefalopatias Metabólicas Congênitas/enzimologia; Encefalopatias Metabólicas Congênitas/genética; Encefalopatias Metabólicas Congênitas/fisiopatologia; Drosophila melanogaster/enzimologia; Glutaratos/metabolismo; Mutação; Domínio Catalítico/genética; Especificidade por Substrato/genética; Estrutura Terciária de Proteína; Proteínas Recombinantes/química; Proteínas Recombinantes/genética; Proteínas Recombinantes/metabolismo

Palavras-chave

2-hydroxyglutaric aciduria; catalytic mechanism; l-2-hydroxyglutarate dehydrogenase; protein crystallography; substrate specificity

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Modelos Moleculares / Encefalopatias Metabólicas Congênitas / Oxirredutases do Álcool / Drosophila melanogaster Limite: Animals / Humans Idioma: En Ano de publicação: 2024 Tipo de documento: Article

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