Biochemical characterization of patients with dihydrolipoamide dehydrogenase deficiency

Dihydrolipoamide dehydrogenase (DLD; E3) oxidizes lipoic acid. Restoring the oxidized state allows lipoic acid to act as a necessary electron sink for the four mitochondrial keto‐acid dehydrogenases: pyruvate dehydrogenase, alpha‐ketoglutarate dehydrogenase, branched‐chain α‐keto‐acid dehydrogenase,...

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Detalles Bibliográficos
Autores principales: Wongkittichote, Parith, Cuddapah, Sanmati R., Master, Stephen R., Grange, Dorothy K., Dietzen, Dennis, Roper, Stephen M., Ganetzky, Rebecca D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Inc. 2023
Materias:
Research Reports
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494496/
https://www.ncbi.nlm.nih.gov/pubmed/37701333
http://dx.doi.org/10.1002/jmd2.12382

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10494496/
https://www.ncbi.nlm.nih.gov/pubmed/37701333
http://dx.doi.org/10.1002/jmd2.12382

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