Upregulation of Bax protein and increased DNA degradation in ALS spinal cord motor neurons

Acta Neurol Scand. 1999 Nov;100(5):317-21. doi: 10.1111/j.1600-0404.1999.tb00403.x.

Abstract

Objectives: To investigate if degeneration of motor neurons in amyotrophic lateral sclerosis (ALS) is related to altered levels of the apoptosis regulating proteins Bcl-2 and Bax. In addition, immunoreactivity of the cysteine protease ICH-IL and detection of motor neurons with DNA fragmentation, indicative of apoptosis, was also studied.

Material and methods: The immunoreactivity of Bcl-2, Bax and ICH-1L were compared in ALS and control spinal cord motor neurons by immunohistochemical analysis and motor neurons with DNA fragmentation were identified by the TUNEL-method.

Results: The results demonstrate an increased expression of Bax in the ALS material as compared to controls but no change in Bcl-2 and ICH-1L expressions. Moreover, a larger proportion of motor neurons stained positive for TUNEL in ALS spinal cords.

Conclusion: Present study suggest an upregulation of the cell death promoting protein Bax and increased DNA degradation, indicative of apoptosis, in spinal motor neurons of ALS patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Amyotrophic Lateral Sclerosis / genetics*
  • Amyotrophic Lateral Sclerosis / physiopathology
  • Apoptosis / genetics
  • DNA / metabolism*
  • Female
  • Genes, bcl-2 / genetics
  • Humans
  • Male
  • Middle Aged
  • Motor Neurons / pathology*
  • Proto-Oncogene Proteins / genetics*
  • Proto-Oncogene Proteins c-bcl-2*
  • Spinal Nerves / pathology*
  • Up-Regulation
  • bcl-2-Associated X Protein

Substances

  • BAX protein, human
  • Proto-Oncogene Proteins
  • Proto-Oncogene Proteins c-bcl-2
  • bcl-2-Associated X Protein
  • DNA