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.

Affiliation

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 in En | MEDLINE | ID: mdl-37995940

Subject(s)

Alcohol Oxidoreductases; Brain Diseases, Metabolic, Inborn; Drosophila melanogaster; Models, Molecular; Animals; Humans; Alcohol Oxidoreductases/chemistry; Alcohol Oxidoreductases/metabolism; Brain Diseases, Metabolic, Inborn/enzymology; Brain Diseases, Metabolic, Inborn/genetics; Brain Diseases, Metabolic, Inborn/physiopathology; Drosophila melanogaster/enzymology; Glutarates/metabolism; Mutation; Catalytic Domain/genetics; Substrate Specificity/genetics; Protein Structure, Tertiary; Recombinant Proteins/chemistry; Recombinant Proteins/genetics; Recombinant Proteins/metabolism

Key words

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

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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Models, Molecular / Brain Diseases, Metabolic, Inborn / Alcohol Oxidoreductases / Drosophila melanogaster Limits: Animals / Humans Language: En Journal: J Biol Chem Year: 2024 Document type: Article

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