Effects of valproic acid on expression of SMN2 mRNA in neuron-like cells derived from patients with spinal muscular atrophy
- VernacularTitle:丙戊酸对脊髓性肌萎缩症患者神经元样细胞SMN2基因mRNA表达的影响
- Author:
Xinming LUO
;
Xiaosu YANG
;
Bo XIAO
- Publication Type:Journal Article
- Keywords:
Muscular atrophy,spinal;
Neurons;
RNA,messenger;
Valproic acid
- From:
Chinese Journal of Neurology
2001;0(03):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the effects of valproic acid (VPA) to the expression of SMN2 mRNA in neuron-like cells (NLCs) derived from patients with spinal muscular atrophy (SMA). Methods Polymerase chain reaction-restriction fragment length polymerphism (PCR-RFLP) was performed to select the patients with SMA. Mesenchymal stem cells (MSCs) derived from patients were induced into NLCs which were set as the model of neurons. The transcripts of SMN2 by reverse transcription-polymerase chain reaction (RT-PCR) combined with sequenceing were detected. Semi-quantitative RT-PCR analysis was performed to detect the changes of SMN2 mRNA expression between before and after the NLCs were treated by VPA. Results Two bands (266 bp and 212 bp) were found in the gel picture of RT-PCR, which were respectively the products of full length transcript (fl-SMN mRNA) and skipping exon 7 (SMN?7 mRNA). NLCs had significantly increased fl-SMN mRNA and SMN?7 mRNA levels as compared with the untreated cells after treatment with VPA, and shown a dose effect(0.210?0.035,0.282?0.041,0.351?0.020,0.450?0.052,0.553?0.035,P
