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Article

Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs.

PloS one - 26 Sept 2007

Gabanella Francesca, Butchbach Matthew E R, Saieva Luciano, Carissimi Claudia, Burghes Arthur H M, Pellizzoni Livio

Abstract excerpt

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 the minor splicing pathways. It is not known whether the levels of spliceosomal snRNPs are decreased in SMA. Here we...

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Source

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Identifiers

PMID: 17895963
DOI: 10.1371/journal.pone.0000921
PMCID: PMC1976558

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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