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Human COQ9 Rescues a coq9 Yeast Mutant by Enhancing Coenzyme Q Biosynthesis from 4-Hydroxybenzoic Acid and Stabilizing the CoQ-Synthome

Coq9 is required for the stability of a mitochondrial multi-subunit complex, termed the CoQ-synthome, and the deamination step of Q intermediates that derive from para-aminobenzoic acid (pABA) in yeast. In human, mutations in the COQ9 gene cause neonatal-onset primary Q(10) deficiency. In this study...

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Detalles Bibliográficos
Autores principales: He, Cuiwen H., Black, Dylan S., Allan, Christopher M., Meunier, Brigitte, Rahman, Shamima, Clarke, Catherine F.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5500610/
https://www.ncbi.nlm.nih.gov/pubmed/28736527
http://dx.doi.org/10.3389/fphys.2017.00463