Ribonucleoprotein Assembly Defects Correlate with Spinal Muscular Atrophy Severity and Preferentially Affect a Subset of Spliceosomal snRNPs

Spinal muscular atrophy (SMA) is a motor neuron disease caused by reduced levels of the survival motor neuron (SMN) protein. SMN together with Gemins2-8 and unrip proteins form a macromolecular complex that functions in the assembly of small nuclear ribonucleoproteins (snRNPs) of both the major and...

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Detalles Bibliográficos
Autores principales: Gabanella, Francesca, Butchbach, Matthew E. R., Saieva, Luciano, Carissimi, Claudia, Burghes, Arthur H. M., Pellizzoni, Livio
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2007
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1976558/
https://www.ncbi.nlm.nih.gov/pubmed/17895963
http://dx.doi.org/10.1371/journal.pone.0000921

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1976558/
https://www.ncbi.nlm.nih.gov/pubmed/17895963
http://dx.doi.org/10.1371/journal.pone.0000921

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