| First Author | Wootz H | Year | 2004 |
| Journal | Biochem Biophys Res Commun | Volume | 322 |
| Issue | 1 | Pages | 281-6 |
| PubMed ID | 15313203 | Mgi Jnum | J:92029 |
| Mgi Id | MGI:3051478 | Doi | 10.1016/j.bbrc.2004.07.118 |
| Citation | Wootz H, et al. (2004) Caspase-12 cleavage and increased oxidative stress during motoneuron degeneration in transgenic mouse model of ALS. Biochem Biophys Res Commun 322(1):281-6 |
| abstractText | Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by loss of motoneurons in the spinal cord and brain stem. We have characterized motoneuron death in transgenic mice carrying the mutant human copper/zinc superoxide dismutase, as a model for familial ALS. Previous studies have shown the involvement of mitochondria in nerve cell demise in these animals. We report here an early cleavage of caspase-12, residing in the endoplasmic reticulum (ER), in the spinal cord during the course of the disease. Apart from caspase-12, caspase-9, and caspase-3 were activated in the transgenic ALS mice. Staining with an antibody for nitrotyrosine, as a marker for oxidative stress, showed a large increase in the ALS mice. The results indicate that oxidative and ER induced stress causing caspase-12 activation are involved in neuronal death and disease progression in ALS. Caspase-12 and the ER pathway for cell death may constitute potential novel targets for the treatment of ALS. |
