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Loss of mitochondrial fatty acid β‐oxidation protein short‐chain Enoyl‐CoA hydratase disrupts oxidative phosphorylation protein complex stability and function

Short‐chain enoyl‐CoA hydratase 1 (ECHS1) is involved in the second step of mitochondrial fatty acid β‐oxidation (FAO), catalysing the hydration of short‐chain enoyl‐CoA esters to short‐chain 3‐hyroxyl‐CoA esters. Genetic deficiency in ECHS1 (ECHS1D) is associated with a specific subset of Leigh Syn...

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Detalles Bibliográficos
Autores principales: Burgin, Harrison, Sharpe, Alice J., Nie, Shuai, Ziemann, Mark, Crameri, Jordan J., Stojanovski, Diana, Pitt, James, Ohtake, Akira, Murayama, Kei, McKenzie, Matthew
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10087869/
https://www.ncbi.nlm.nih.gov/pubmed/35962613
http://dx.doi.org/10.1111/febs.16595