Bcl-x pre-mRNA splicing regulates brain injury after neonatal hypoxia-ischemia

J Neurosci. 2012 Sep 26;32(39):13587-96. doi: 10.1523/JNEUROSCI.2617-12.2012.

Abstract

The bcl-x gene appears to play a critical role in regulating apoptosis in the developing and mature CNS and following CNS injury. Two isoforms of Bcl-x are produced as a result of alternative pre-mRNA splicing: Bcl-x(L) (the long form) is anti-apoptotic, while Bcl-x(S) (short form) is pro-apoptotic. Despite the antagonistic activities of these two isoforms, little is known about how regulation of alternative splicing of bcl-x may mediate neural cell apoptosis. Here, we report that apoptotic stimuli (staurosporine or C2-ceramide) reciprocally altered Bcl-x splicing in neural cells, decreasing Bcl-x(L) while increasing Bcl-x(S). Specific knockdown of Bcl-x(S) attenuated apoptosis. To further define regulatory elements that influenced Bcl-x splicing, a Bcl-x minigene was constructed. Deletional analysis revealed several consensus sequences within intron 2 that altered splicing. We found that the splicing factor, CUG-binding-protein-1 (CUGBP1), bound to a consensus sequence close to the Bcl-x(L) 5' splice site, altering the Bcl-x(L)/Bcl-x(S) ratio and influencing cell death. In vivo, neonatal hypoxia-ischemia reciprocally altered Bcl-x pre-mRNA splicing, similar to the in vitro studies. Manipulation of the splice isoforms using viral gene transfer of Bcl-x(S) shRNA into the hippocampus of rats before neonatal hypoxia-ischemia decreased vulnerability to injury. Moreover, alterations in nuclear CUGBP1 preceded Bcl-x splicing changes. These results suggest that alternative pre-mRNA splicing may be an important regulatory mechanism for cell death after acute neurological injury and may potentially provide novel targets for intervention.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alternative Splicing / genetics*
  • Analysis of Variance
  • Animals
  • Animals, Newborn
  • Apoptosis / drug effects
  • Apoptosis / genetics
  • Brain Injuries / etiology*
  • CELF1 Protein
  • Cells, Cultured
  • Cerebral Cortex / cytology
  • Dose-Response Relationship, Drug
  • Embryo, Mammalian
  • Enzyme Inhibitors / pharmacology
  • Female
  • Functional Laterality
  • Gene Expression Regulation / drug effects
  • Gene Expression Regulation / genetics
  • Green Fluorescent Proteins / genetics
  • Humans
  • Hypoxia-Ischemia, Brain / complications*
  • In Situ Nick-End Labeling
  • L-Lactate Dehydrogenase / metabolism
  • Male
  • Mice
  • Neurons / drug effects
  • Neurons / metabolism
  • Pregnancy
  • RNA Precursors / genetics
  • RNA Precursors / metabolism*
  • RNA, Small Interfering / metabolism
  • RNA-Binding Proteins / metabolism
  • Rats
  • Sphingosine / analogs & derivatives
  • Sphingosine / pharmacology
  • Staurosporine / pharmacology
  • Time Factors
  • Transfection / methods
  • bcl-X Protein / genetics
  • bcl-X Protein / metabolism*

Substances

  • CELF1 Protein
  • Celf1 protein, rat
  • Enzyme Inhibitors
  • N-acetylsphingosine
  • RNA Precursors
  • RNA, Small Interfering
  • RNA-Binding Proteins
  • bcl-X Protein
  • enhanced green fluorescent protein
  • Green Fluorescent Proteins
  • L-Lactate Dehydrogenase
  • Staurosporine
  • Sphingosine