| Literature DB >> 31102025 |
Shiori Ando1,2, Michinori Funato2, Kazuki Ohuchi1,2, Satoshi Inagaki1,2, Arisu Sato1,2, Junko Seki2, Chizuru Kawase2, Toshio Saito3, Hisahide Nishio4, Shinsuke Nakamura1, Masamitsu Shimazawa1, Hideo Kaneko2, Hideaki Hara5.
Abstract
Spinal muscular atrophy (SMA) is an inherited disease characterized by progressive motor neuron death and subsequent muscle weakness and is caused by deletion or mutation of survival motor neuron (SMN) 1 gene. Protecting spinal motor neuron is an effective clinical strategy for SMA. The purpose of this study was to investigate the potential effect of an anti-epileptic drug levetiracetam on SMA. In the present study, we used differentiated spinal motor neurons (MNs) from SMA patient-derived induced pluripotent stem cells (SMA-iPSCs) to investigate the effect of levetiracetam. Levetiracetam promoted neurite elongation in SMA-iPSCs-MNs. TUNEL-positive spinal motor neurons were significantly reduced by levetiracetam in SMA-iPSCs-MNs. In addition, the expression level of cleaved-caspase 3 was decreased by levetiracetam in SMA-iPSCs-MNs. Furthermore, levetiracetam improved impaired mitochondrial function in SMA-iPSCs-MNs. On the other hand, levetiracetam did not affect the expression level of SMN protein in SMA-iPSCs-MNs. These findings indicate that levetiracetam has a neuroprotective effect for SMA.Entities:
Keywords: Induced pluripotent stem cells; Levetiracetam; Motor neuron; Spinal muscular atrophy
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Year: 2019 PMID： 31102025 DOI： 10.1007/s11064-019-02814-4
Source DB: PubMed Journal: Neurochem Res ISSN： 0364-3190 Impact factor: 3.996