| First Author | Kira Y | Year | 2006 |
| Journal | Brain Res | Volume | 1070 |
| Issue | 1 | Pages | 206-14 |
| PubMed ID | 16412993 | Mgi Jnum | J:105888 |
| Mgi Id | MGI:3616934 | Doi | 10.1016/j.brainres.2005.11.052 |
| Citation | Kira Y, et al. (2006) l-Carnitine suppresses the onset of neuromuscular degeneration and increases the life span of mice with familial amyotrophic lateral sclerosis. Brain Res 1070(1):206-14 |
| abstractText | Amyotrophic lateral sclerosis (ALS) is a fatal disease caused by progressive degeneration of motor neurons in the spinal cord and motor cortex. Although the etiology of ALS remains unknown, a mutation of the gene encoding Cu,Zn-superoxide dismutase (SOD1) has been reported in 20% of familial cases of ALS (FALS). Transgenic mice that overexpress a mutated human SOD1 exhibit a phenotype and pathology similar to those observed in patients with FALS. Mitochondrial abnormality has been reported in patients with ALS and in animal models of FALS. We recently reported that l-carnitine, an essential cofactor for the beta-oxidation of long-chain fatty acids, effectively inhibits various types of mitochondrial injury and apoptosis both in vitro and in vivo. The present study demonstrates that oral administration of l-carnitine prior to disease onset significantly delayed the onset of signs of disease (log-rank P = 0.0008), delayed deterioration of motor activity, and extended life span (log-rank P = 0.0001) in transgenic mice carrying a human SOD1 gene with a G93A mutation (Tg). More importantly, subcutaneous injection of l-carnitine increased the life span of Tg mice (46% increase in male, 60% increase in female) even when given after the appearance of signs of disease. |
