| Budget Amount *help |
¥18,850,000 (Direct Cost: ¥14,500,000、Indirect Cost: ¥4,350,000)
Fiscal Year 2014: ¥6,110,000 (Direct Cost: ¥4,700,000、Indirect Cost: ¥1,410,000)
Fiscal Year 2013: ¥5,980,000 (Direct Cost: ¥4,600,000、Indirect Cost: ¥1,380,000)
Fiscal Year 2012: ¥6,760,000 (Direct Cost: ¥5,200,000、Indirect Cost: ¥1,560,000)
|
|---|
| Outline of Final Research Achievements |
TDP-43 gene has been identified as a causative gene of amyotrophic lateral sclerosis (ALS) . Two types of mutant TDP-43 knock-in (mTDP-43 KI) mice with mutations in different sites of the gene were generated to investigate the biological effects of each mutation. Considerable phenotypes were observed in mTDP-43 KI mice: poor weight gain and loss of spinal motor neurons, which is related to ALS symptoms. By Exon Array and RT-PCR, defects of RNA metabolism following TDP-43 dysfunction were detected in the mononuclear cells from peripheral blood of KI mice. Furthermore, up-regulation of mutant TDP-43 was detected in astrocytes adjacent to motor neurons from KI mice. Our results, referencing TDP-43 mutations, will provide new insights into the pathophysiology of ALS.
|
