Gestational Age-Dependent Increase of Survival Motor Neuron Protein in Umbilical Cord-Derived Mesenchymal Stem Cells
| العنوان: | Gestational Age-Dependent Increase of Survival Motor Neuron Protein in Umbilical Cord-Derived Mesenchymal Stem Cells |
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| المؤلفون: | Kazumichi Fujioka, Kazumoto Iijima, Nur Imma Fatimah Harahap, Daisuke Kurokawa, Sota Iwatani, Makiko Yoshida, Khin Kyae Mon Thwin, Tsubasa Koda, Akemi Shono, Shinya Tairaku, Keiji Yamana, Hisahide Nishio, Ichiro Morioka, Hideto Yamada, Noriyuki Nishimura, Mariko Taniguchi-Ikeda, Masami Mizobuchi, Dian Kesumapramudya Nurputra |
| المصدر: | Frontiers in Pediatrics Frontiers in Pediatrics, Vol 5 (2017) |
| سنة النشر: | 2017 |
| مصطلحات موضوعية: | 0301 basic medicine, medicine.drug_class, animal diseases, survival motor neuron-targeted therapy, Umbilical cord, Pediatrics, Andrology, 03 medical and health sciences, medicine, gestational age, Original Research, spinal muscular atrophy, Messenger RNA, Fetus, business.industry, Histone deacetylase inhibitor, Mesenchymal stem cell, lcsh:RJ1-570, lcsh:Pediatrics, Spinal muscular atrophy, Motor neuron, medicine.disease, SMA, nervous system diseases, 030104 developmental biology, medicine.anatomical_structure, nervous system, umbilical cord-derived mesenchymal stem cell, Pediatrics, Perinatology and Child Health, Immunology, business, perinatal development |
| الوصف: | Background: Spinal muscular atrophy (SMA) is the most common genetic neurological disease leading to infant death. It is caused by loss of survival motor neuron (SMN) 1 gene and subsequent reduction of SMN protein in motor neurons. Because SMN is ubiquitously expressed and functionally linked to general RNA metabolism pathway, fibroblasts are most widely used for the assessment of SMN expression in SMA patients but usually isolated from skin biopsy samples after the onset of overt symptoms. Although recent translational studies of SMN-targeted therapies have revealed the very limited time-window for effective SMA therapies during perinatal period, the exact time-point when SMN shortage became evident is unknown in human samples. In the present study, we analyzed SMN mRNA and protein expression during perinatal period by using umbilical cord-derived mesenchymal stem cells (UC-MSCs) obtained from preterm and term infants. Methods: UC-MSCs were isolated from 16 control infants delivered at 22-40 weeks of gestation and SMA fetus aborted at 19 weeks of gestation (UC-MSC-Control and UC-MSC-SMA). Fibroblasts were isolated from control volunteer and SMA patient (FB-Control and FB-SMA). SMN mRNA and protein expression in UC-MSCs and fibroblasts was determined by RT-qPCR and Western blot. Results: UC-MSC-Control and UC-MSC-SMA expressed the comparable level of MSC markers on their cell surface, and were able to differentiate into adipocytes, osteocytes, and chondrocytes. At steady state, SMN mRNA and protein expression was decreased in UC-MSC-SMA compared to UC-MSC-Control, as observed in FB-SMA and FB-Control. In response to histone deacetylase inhibitor valproic acid, SMN mRNA and protein expression in UC-MSC-SMA as well as FB-SMA was increased. During perinatal development from 22 to 40 weeks of gestation, SMN mRNA and protein expression in UC-MSC-Control was positively correlated with gestational age. Conclusions: UC-MSCs isolated from 17 fetus/infant of 19-40 weeks of gestation are expressed functional SMN mRNA and protein. SMN mRNA and protein expression in UC-MSCs is increased with gestational age during perinatal development. |
| وصف الملف: | application/pdf |
| تدمد: | 2296-2360 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e6aebdbe3d67d7edd535a7a967c74fd8 https://pubmed.ncbi.nlm.nih.gov/28929094 |
| Rights: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....e6aebdbe3d67d7edd535a7a967c74fd8 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 22962360 |
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