Research

Research ›› 2025, Vol. 8 ›› Issue (5) : 0661 . DOI: 10.34133/research.0661

Research Article

Structural Insights into Isovaleryl-Coenzyme A Dehydrogenase: Mechanisms of Substrate Specificity and Implications of Isovaleric Acidemia-Associated Mutations

Kaide Ju ¹^,^† ,
Fang Bai ²^,^† ,
Youwei Xu ³^,^† ,
Qingao Li ² ,
Gengchen Su ¹ ,
Ye Jin ⁴ ,
Houzao Chen ⁵^,^* ,
Shuyang Zhang ¹^,²^,⁴^,⁶^,^* ,
Xiaodong Luan ⁴^,⁷^,^*

Author information +

¹ School of Basic Medical Sciences, Tsinghua University, Beijing 100084, China.

² Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China.

³ The CAS Key Laboratory of Receptor Research, Shanghai Institute of Materia Medica, Chinese Academy of Sciences, Shanghai 201203, China.

⁴ Department of Rare Diseases, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Beijing 100730, China.

⁵ Department of Biochemistry and Molecular Biology, State Key Laboratory of Medical Molecular Biology, Institute of Basic Medical Sciences, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China.

⁶ Tsinghua-Peking Center for Life Sciences, Tsinghua University, Beijing 100084, China.

⁷ Center for Drug Research and Evaluation, Institute of Clinical Medicine, Peking Union Medical College Hospital, Beijing 100730, China.

^* Address correspondence to: luanxiaodong@pumch.cn (X.L.); shuyangzhang103@nrdrs.org (S.Z.); chenhouzao@ibms.cams.cn (H.C.)

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Received	Accepted	Published
2024-09-28	2025-03-12
Issue Date
2025-07-23

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