| Budget Amount *help |
¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2013: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2012: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2011: ¥2,730,000 (Direct Cost: ¥2,100,000、Indirect Cost: ¥630,000)
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| Research Abstract |
Higher levels of 4-Hydroxynonenal (HNE) have been shown in the cerebrospinal fluid of amyotrophic lateral sclerosis (ALS) patients and mice models of ALS. In this study, we investigated phathophysiological roles of HNE in ALS and protective potentials of N-acetyl-L-cysteine (NAC) and its derivative, NAC-amide (NACA) in a mouse model of ALS (G93A). The levels of HNE-modified proteins in the spinal cord of the G93A were significantly increased at 15 weeks (onset of motor disturbance) and older. Administration of NAC had no effect on disease progression and survival in the G93A. In contrast, administration of NACA slowed disease progression and prolonged survival in these mice. These results suggest that HNE may play a role in the degeneration of motoneurons in the G93A, and that NACA is a promising therapeutic drug for ALS.
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