LRPPRC and SLIRP Interact in a Ribonucleoprotein Complex That Regulates Posttranscriptional Gene Expression in Mitochondria

Mutations in LRPPRC are responsible for the French Canadian variant of Leigh syndrome (LSFC), a neurodegenerative disorder caused by a tissue-specific deficiency in cytochrome c oxidase (COX). To investigate the pathogenic mechanism of disease, we studied LRPPRC function in LSFC and control fibrobla...

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Detalles Bibliográficos
Autores principales: Sasarman, Florin, Brunel-Guitton, Catherine, Antonicka, Hana, Wai, Timothy, Shoubridge, Eric A.
Formato: Texto
Lenguaje:English
Publicado: The American Society for Cell Biology 2010
Materias:
Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2854090/
https://www.ncbi.nlm.nih.gov/pubmed/20200222
http://dx.doi.org/10.1091/mbc.E10-01-0047

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2854090/
https://www.ncbi.nlm.nih.gov/pubmed/20200222
http://dx.doi.org/10.1091/mbc.E10-01-0047

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