Defective function of α-ketoglutarate dehydrogenase exacerbates mitochondrial ATP deficits during complex I deficiency

The NDUFS4 knockout (KO) mouse phenotype resembles the human Complex I deficiency Leigh Syndrome. The irreversible succination of protein thiols by fumarate is increased in select regions of the NDUFS4 KO brain affected by neurodegeneration. We report that dihydrolipoyllysine-residue succinyltransfe...

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Detalles Bibliográficos
Autores principales: Piroli, Gerardo G., Manuel, Allison M., McCain, Richard S., Smith, Holland H., Ozohanics, Oliver, Mellid, Sara, Cox, J. Hunter, Cotham, William E., Walla, Michael D., Cascón, Alberto, Ambrus, Attila, Frizzell, Norma
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2023
Materias:
Research Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10618796/
https://www.ncbi.nlm.nih.gov/pubmed/37883842
http://dx.doi.org/10.1016/j.redox.2023.102932

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10618796/
https://www.ncbi.nlm.nih.gov/pubmed/37883842
http://dx.doi.org/10.1016/j.redox.2023.102932

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