Abolishing Trp53-dependent apoptosis does not benefit spinal muscular atrophy model mice

Ming S. Tsai, Yung T. Chiu, Sue H. Wang, Hsiu M. Hsieh-Li, Hung Li*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

10 Citations (Scopus)


Spinal muscular atrophy (SMA) is the most common genetic motoneuron degenerative disorder, but the mechanism(s) of motoneuron death is unclear. Previously, a direct interaction between tumor-suppressive TP53 protein and the SMA determinant gene product, survival motor neuron protein, was identified and therefore it has been suggested that a mechanism of TP53-dependent apoptosis plays an important role in motoneuron degeneration in SMA. We used our SMA model mice, generated by a combination of knockout and transgenic techniques, to decipher the role of TP53 protein in the motoneuron degeneration in SMA. We detected a significant increase of Trp53 expression in the spinal cord of SMA-like mice compared to their normal littermates. After crossing SMA-like mice with Trp53 knockout mice, the progeny Trp53-deficient SMA-like mice did not show milder disease severity or longer lifespan compared to SMA littermates with wild-type Trp53 genes. Our studies provide in vivo evidence indicating that Trp53-dependent apoptosis does not play a crucial role in motoneuron degeneration in SMA-like mice.

Original languageEnglish
Pages (from-to)372-375
Number of pages4
JournalEuropean Journal of Human Genetics
Issue number3
Publication statusPublished - 2006 Mar


  • Motoneuron degeneration
  • SMA model mice
  • Spinal muscular atrophy
  • Trp53-dependent apoptosis

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)


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