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https://hdl.handle.net/20.500.14094/90004406
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2024-04-24
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90004406 (fulltext)
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90004406
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open access
出版タイプ
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タイトル
Gestational Age-Dependent Increase of Survival Motor Neuron Protein in Umbilical Cord-Derived Mesenchymal Stem Cells
著者
Iwatani, Sota ; Harahap, Nur Imma Fatimah ; Nurputra, Dian Kesumapramudya ; Tairaku, Shinya ; Shono, Akemi ; Kurokawa, Daisuke ; Yamana, Keiji ; Thwin, Khin Kyae Mon ; Yoshida, Makiko ; Mizobuchi, Masami ; Koda, Tsubasa ; Fujioka, Kazumichi ; Taniguchi-Ikeda, Mariko ; Yamada, Hideto ; Morioka, Ichiro ; Iijima, Kazumoto ; Nishio, Hisahide ; Nishimura, Noriyuki
著者ID
A0851
研究者ID
1000000741430
著者名
Iwatani, Sota
岩谷, 壮太
イワタニ, ソウタ
所属機関名
医学部附属病院
著者名
Harahap, Nur Imma Fatimah
著者名
Nurputra, Dian Kesumapramudya
著者ID
A1457
研究者ID
1000030618781
著者名
Tairaku, Shinya
平久, 進也
タイラク, シンヤ
所属機関名
医学部附属病院
著者ID
A0189
研究者ID
1000010535066
著者名
Shono, Akemi
庄野, 朱美
ショウノ, アケミ
所属機関名
医学研究科
著者ID
A1529
研究者ID
1000050769750
著者名
Kurokawa, Daisuke
黒川, 大輔
クロカワ, ダイスケ
所属機関名
医学部附属病院
著者ID
A1550
研究者ID
1000080792936
著者名
Yamana, Keiji
山名, 啓司
ヤマナ, ケイジ
所属機関名
医学部附属病院
著者名
Thwin, Khin Kyae Mon
著者名
Yoshida, Makiko
著者名
Mizobuchi, Masami
著者名
Koda, Tsubasa
著者ID
A0863
研究者ID
1000020568810
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail?systemId=61a996a431fe1218520e17560c007669
著者名
Fujioka, Kazumichi
藤岡, 一路
フジオカ, カズミチ
所属機関名
医学部附属病院
著者名
Taniguchi-Ikeda, Mariko
著者ID
A0041
研究者ID
1000040220397
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail?systemId=d4626745e9403360520e17560c007669
著者名
Yamada, Hideto
山田, 秀人
ヤマダ, ヒデト
所属機関名
医学研究科
著者ID
A1383
研究者ID
1000080437467
著者名
Morioka, Ichiro
森岡, 一朗
モリオカ, イチロウ
所属機関名
医学研究科
著者ID
A0877
研究者ID
1000000240854
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail?systemId=1a50a9148347284a520e17560c007669
著者名
Iijima, Kazumoto
飯島, 一誠
イイジマ, カヅモト
所属機関名
医学研究科
著者ID
A0756
研究者ID
1000080189258
著者名
Nishio, Hisahide
西尾, 久英
ニシオ, ヒサヒデ
所属機関名
医学研究科
著者ID
A1683
研究者ID
1000000322719
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail?systemId=435041c825fb64d4520e17560c007669
著者名
Nishimura, Noriyuki
西村, 範行
ニシムラ, ノリユキ
所属機関名
保健学研究科
収録物名
Frontiers in Pediatrics
巻(号)
5
ページ
194-194
出版者
Frontiers Media
刊行日
2017-09-05
公開日
2017-12-21
抄録
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 (FBs) 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 this 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). FBs were isolated from control volunteer and SMA patient (FB-Control and FB-SMA). SMN mRNA and protein expression in UC-MSCs and FBs 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 and 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. Conclusion: 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.
キーワード
spinal muscular atrophy
survival motor neuron-targeted therapy
umbilical cord-derived mesenchymal stem cell
gestational age
perinatal development
カテゴリ
医学研究科
医学部附属病院
保健学研究科
学術雑誌論文
権利
This Document is Protected by copyright and was first published by Frontiers. All rights reserved. it is reproduced with permission. © 2017 Iwatani, Harahap, Nurputra, Tairaku, Shono, Kurokawa, Yamana, Thwin, Yoshida, Mizobuchi, Koda, Fujioka, Taniguchi-Ikeda, Yamada, Morioka, Iijima, Nishio and Nishimura.
This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
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資源タイプ
journal article
言語
English (英語)
eISSN
2296-2360
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関連情報
DOI
https://doi.org/10.3389/fped.2017.00194
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