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Altered RNA metabolism due to a homozygous RBM7 mutation in a patient with spinal motor neuropathy

The exosome complex is the most important RNA processing machinery within the cell. Mutations in its subunits EXOSC8 and EXOSC3 cause pontocerebellar hypoplasia, spinal muscular atrophy (SMA) and central nervous system demyelination. We present a patient with SMA-like phenotype carrying a homozygous...

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Detalhes bibliográficos
Publicado no:Hum Mol Genet
Main Authors: Giunta, Michele, Edvardson, Shimon, Xu, Yaobo, Schuelke, Markus, Gomez-Duran, Aurora, Boczonadi, Veronika, Elpeleg, Orly, Müller, Juliane S., Horvath, Rita
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2016
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5181591/
https://ncbi.nlm.nih.gov/pubmed/27193168
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddw149
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