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TitleStructural and biochemical mechanism of short-chain enoyl-CoA hydratase (ECHS1) substrate recognition.
Journal, issue, pagesCommun Biol, Vol. 8, Issue 1, Page 619, Year 2025
Publish dateApr 16, 2025
AuthorsGengchen Su / Youwei Xu / Binxian Chen / Kaide Ju / Ye Jin / Houzao Chen / Shuyang Zhang / Xiaodong Luan /
PubMed AbstractDeficiency of short-chain enoyl-CoA hydratase (ECHS1), a crucial enzyme in fatty acid metabolism through the mitochondrial β-oxidation pathway, has been strongly linked to various diseases, ...Deficiency of short-chain enoyl-CoA hydratase (ECHS1), a crucial enzyme in fatty acid metabolism through the mitochondrial β-oxidation pathway, has been strongly linked to various diseases, especially cardiomyopathy. However, the structural and biochemical mechanisms through which ECHS1 recognizes acyl-CoAs remain poorly understood. Herein, cryo-EM analysis reveals the apo structure of ECHS1 and structures of the ECHS1-crotonyl-CoA, ECHS1-acetoacetyl-CoA, ECHS1-hexanoyl-CoA, and ECHS1-octanoyl-CoA complexes at high resolutions. The mechanism through which ECHS1 recognizes its substrates varies with the fatty acid chain lengths of acyl-CoAs. Furthermore, crucial point mutations in ECHS1 have a great impact on substrate recognition, resulting in significant changes in binding affinity and enzyme activity, as do disease-related point mutations in ECHS1. The functional mechanism of ECHS1 is systematically elucidated from structural and biochemical perspectives. These findings provide a theoretical basis for subsequent work focused on determining the role of ECHS1 deficiency (ECHS1D) in the occurrence of diseases such as cardiomyopathy.
External linksCommun Biol / PubMed:40240482 / PubMed Central
MethodsEM (single particle)
Resolution2.18 - 2.55 Å
Structure data

60405

8zru

EMDB-60405, PDB-8zru:
Structure of human ECHS1 in apo state
Method: EM (single particle) / Resolution: 2.18 Å

60406

8zrv

EMDB-60406, PDB-8zrv:
Structure of human ECHS1 in complex with Hexanoyl-CoA
Method: EM (single particle) / Resolution: 2.55 Å

60407

8zrw

EMDB-60407, PDB-8zrw:
Structure of human ECHS1 in complex with Octanoyl-CoA
Method: EM (single particle) / Resolution: 2.29 Å

60408

8zrx

EMDB-60408, PDB-8zrx:
Structure of human ECHS1 in complex with Acetoacetyl-CoA
Method: EM (single particle) / Resolution: 2.27 Å

60409

8zry

EMDB-60409, PDB-8zry:
Structure of human ECHS1 in complex with Crotonoyl-CoA
Method: EM (single particle) / Resolution: 2.23 Å

Chemicals

ChemComp-MG:
Unknown entry

ChemComp-HXC:
HEXANOYL-COENZYME A

ChemComp-CO8:
OCTANOYL-COENZYME A

ChemComp-HOH:
WATER

ChemComp-CAA:
ACETOACETYL-COENZYME A

PDB-1d88:
CONFORMATIONAL INFLUENCE OF THE RIBOSE 2'-HYDROXYL GROUP: CRYSTAL STRUCTURES OF DNA-RNA CHIMERIC DUPLEXES

Source
  • homo sapiens (human)
KeywordsHYDROLASE / ECHS1 / Hexanoyl-CoA / Octanoyl-CoA / Acetoacetyl-CoA / Crotonoyl-CoA

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