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Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders

BACKGROUND: Defects in genes involved in mitochondrial fatty-acid oxidation (mFAO) reduce the ability of patients to cope with metabolic challenges. mFAO enzymes accept multiple substrates of different chain length, leading to molecular competition among the substrates. Here, we combined computation...

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Detalles Bibliográficos
Autores principales: van Eunen, Karen, Volker-Touw, Catharina M. L., Gerding, Albert, Bleeker, Aycha, Wolters, Justina C., van Rijt, Willemijn J., Martines, Anne-Claire M. F., Niezen-Koning, Klary E., Heiner, Rebecca M., Permentier, Hjalmar, Groen, Albert K., Reijngoud, Dirk-Jan, Derks, Terry G. J., Bakker, Barbara M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5142382/
https://www.ncbi.nlm.nih.gov/pubmed/27927213
http://dx.doi.org/10.1186/s12915-016-0327-5