title: [An experimental research on differentiation of mesenchymal stem cells derived from children with spinal muscular atrophy into neuron-like cells].
abstract: OBJECTIVE
  Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease. It is characterized by selective loss of spinal cord motor neurons leading to muscle atrophy and is the result of mutation or deletion of the survival motor neuron (SMN) gene. Currently, there are no effective therapies for this disease. Stem cell therapy is a new prospect for SMA patients. This study aimed to investigate whether mesenchymal stem cells (MSCs) can be differentiated into neuron-like cells (NLCs) in SMA patients in order to provide a basis for stem cell therapy for SMA.
  
  
  METHODS
  SMA was definitively diagnosed using polymerase chain reaction-restriction fragment length polymerphhism (PCR-RFLP). Two children without SMN1 gene deletion were used as controls. MSCs were isolated and purified from SMA patients and controls, and induced into NLCs by bFGF and baicalin. The NLCs were identified by immunofluourescence staining with NSE and NF monoclonal antibodies.
  
  
  RESULTS
  SMA patients showed the deletion of SMN1 exon 7. The morphous and proliferative speed of MSCs between SMA patients and controls were similar. After 6-day induction, MSCs of the two groups displayed similar morphology to that of neurons, with long processes forming extensive networks. NSE and NF, the neuronal markers, were detected in the differentiated NLCs of the two groups.
  
  
  CONCLUSIONS
  SMN1 deletion appears not to affect the proliferation and differentiation of MSCs. MSCs of SMA patients can be differentiated into NLCs.
authors.affiliation: Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, China. yanxia@public.cs.hn.cn
authors.forename: Xiao-Su
authors.initials: XS
authors.lastname: Yang
authors.forename: Xin-Ming
authors.initials: XM
authors.lastname: Luo
authors.forename: Bo
authors.initials: B
authors.lastname: Xiao
authors.forename: Xin-Zhong
authors.initials: XZ
authors.lastname: Li
mesh_terms: D000293:Adolescent; D002454:Cell Differentiation; D049109:Cell Proliferation; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D059630:Mesenchymal Stem Cells / Q000166:cytology*; D009134:Muscular Atrophy, Spinal / Q000473:pathology*; D009474:Neurons / Q000166:cytology*