SMN interacts with a novel family of hnRNP and spliceosomal proteins

Zissimos Mourelatos, Linda Abel, Jeongsik Yong, Naoyuki Kataoka, Gideon Dreyfuss

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173 Scopus citations


Spinal muscular atrophy (SMA) is a common neuro-degenerative disease caused by deletion or loss-of-function mutations of the survival of motor neurons (SMN) protein. SMN is in a complex with several proteins, including Gemin2, Gemin3 and Gemin4, and it plays important roles in small nuclear ribonucleo-protein (snRNP) biogenesis and in pre-mRNA splicing. Here, we characterize three new hnRNP proteins, collectively referred to as hnRNP Qs, which are derived from alternative splicing of a single gene. The hnRNP Q proteins interact with SMN, and the most common SMN mutant found in SMA patients is defective in its interactions with them. We further demonstrate that hnRNP Qs are required for efficient pre-mRNA splicing in vitro. The hnRNP Q proteins may provide a molecular link between the SMN complex and splicing.

Original languageEnglish (US)
Pages (from-to)5443-5452
Number of pages10
JournalEMBO Journal
Issue number19
StatePublished - Oct 1 2001


  • HnRNP proteins
  • Pre-mRNA splicing
  • Spinal muscular atrophy
  • Survival of motor neurons


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