Interaction of hnRNPA1/A2 and DAZAP1 with an Alu-Derived Intronic Splicing Enhancer Regulates ATM Aberrant Splicing
2011

How Certain Proteins Affect Splicing of a Gene Related to Ataxia

publication Evidence: moderate

Author Information

Author(s): Pastor Tibor Pagani, Franco Cotterill

Primary Institution: International Centre for Genetic Engineering and Biotechnology, Trieste, Italy

Hypothesis

The interaction of hnRNPA1/A2 and DAZAP1 with an Alu-derived intronic splicing enhancer regulates ATM aberrant splicing.

Conclusion

The study identifies hnRNPA1/A2 as a negative regulator and DAZAP1 as a positive regulator of cryptic exon inclusion in the ATM gene.

Supporting Evidence

  • hnRNPA1 negatively affects splicing selection while DAZAP1 positively influences it.
  • Depletion of hnRNPA1 leads to increased cryptic exon inclusion.
  • DAZAP1 knockdown results in a modest increase in cryptic exon exclusion.

Takeaway

Some proteins can help or hurt the process of making a gene's final message, which can affect how diseases like ataxia develop.

Methodology

The study used RNA pull-down assays and functional studies with overexpression and siRNA experiments to analyze splicing factor interactions.

Digital Object Identifier (DOI)

10.1371/journal.pone.0023349

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