Inefficient Thermogenic Mitochondrial Respiration Due to Futile Proton Leak in a Mouse Model of Fragile X Syndrome

Fragile X syndrome (FXS) is the leading known inherited intellectual disability and the most common genetic cause of autism. The full mutation results in transcriptional silencing of the Fmr1 gene and loss of fragile X mental retardation protein (FMRP) expression. Defects in neuroenergetic capacity...

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Detalhes bibliográficos
Publicado no:FASEB J
Main Authors: Griffiths, Keren K., Wang, Aili, Wang, Lifei, Tracey, Matthew, Kleiner, Giulio, Quinzii, Catarina M., Sun, Linlin, Yang, Guang, Perez-Zoghbi, Jose F., Licznerski, Pawel, Yang, Mu, Jonas, Elizabeth A., Levy, Richard J.
Formato: Artigo
Idioma:Inglês
Publicado em: 2020
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7692004/
https://ncbi.nlm.nih.gov/pubmed/32307754
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.202000283RR
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