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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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Главные авторы: | , , , , , |
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Формат: | Artigo |
Язык: | Inglês |
Опубликовано: |
Public Library of Science
2007
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Предметы: | |
Online-ссылка: | https://ncbi.nlm.nih.gov/pmc/articles/PMC1976558/ https://ncbi.nlm.nih.gov/pubmed/17895963 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0000921 |
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