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

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

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
Volume14
Issue number3
DOIs
Publication statusPublished - 2006 Mar 1

Fingerprint

Spinal Muscular Atrophy
Apoptosis
Motor Neurons
Tumor Suppressor Protein p53
Knockout Mice
Genes
Spinal Cord

Keywords

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

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

Cite this

Abolishing Trp53-dependent apoptosis does not benefit spinal muscular atrophy model mice. / Tsai, Ming S.; Chiu, Yung T.; Wang, Sue H.; Hsieh-Li, Hsiu M.; Li, Hung.

In: European Journal of Human Genetics, Vol. 14, No. 3, 01.03.2006, p. 372-375.

Research output: Contribution to journalArticle

Tsai, Ming S. ; Chiu, Yung T. ; Wang, Sue H. ; Hsieh-Li, Hsiu M. ; Li, Hung. / Abolishing Trp53-dependent apoptosis does not benefit spinal muscular atrophy model mice. In: European Journal of Human Genetics. 2006 ; Vol. 14, No. 3. pp. 372-375.
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