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MiR-375 is Essential for Human Spinal Motor Neuron Development and May Be Involved in Motor Neuron Degeneration

Title: MiR-375 is Essential for Human Spinal Motor Neuron Development and May Be Involved in Motor Neuron Degeneration
Authors: Bhinge, Akshay; Namboori, Seema C.; Bithell, Angela; Soldati, Chiara; Buckley, Noel J.; Stanton, Lawrence W.
Contributors: A*STAR, Singapore
Source: Stem Cells ; volume 34, issue 1, page 124-134 ; ISSN 1066-5099 1549-4918
Publisher Information: Oxford University Press (OUP)
Publication Year: 2015
Description: The transcription factor REST is a key suppressor of neuronal genes in non-neuronal tissues. REST has been shown to suppress proneuronal microRNAs in neural progenitors indicating that REST-mediated neurogenic suppression may act in part via microRNAs. We used neural differentiation of Rest-null mouse ESC to identify dozens of microRNAs regulated by REST during neural development. One of the identified microRNAs, miR-375, was upregulated during human spinal motor neuron development. We found that miR-375 facilitates spinal motor neurogenesis by targeting the cyclin kinase CCND2 and the transcription factor PAX6. Additionally, miR-375 inhibits the tumor suppressor p53 and protects neurons from apoptosis in response to DNA damage. Interestingly, motor neurons derived from a spinal muscular atrophy patient displayed depressed miR-375 expression and elevated p53 protein levels. Importantly, SMA motor neurons were significantly more susceptible to DNA damage induced apoptosis suggesting that miR-375 may play a protective role in motor neurons.
Document Type: article in journal/newspaper
Language: English
DOI: 10.1002/stem.2233
Availability: https://doi.org/10.1002/stem.2233; https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1002%2Fstem.2233; https://onlinelibrary.wiley.com/doi/pdf/10.1002/stem.2233; https://onlinelibrary.wiley.com/doi/full-xml/10.1002/stem.2233; https://academic.oup.com/stmcls/article-pdf/34/1/124/42026081/stmcls_34_1_124.pdf
Rights: https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model
Accession Number: edsbas.FCDF6613
Database: BASE