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Open AccessArticle

Agenesis and Hypomyelination of Corpus Callosum in Mice Lacking Nsun5, an RNA Methyltransferase

1
State Key Laboratory of Reproductive Medicine, Department of Physiology, Nanjing Medical University, Nanjing 211166, China
2
Department of Physiology, Nanjing Medical University, Nanjing 211166, China
*
Authors to whom correspondence should be addressed.
These authors contributed equally to this work.
Cells 2019, 8(6), 552; https://doi.org/10.3390/cells8060552
Received: 19 May 2019 / Revised: 3 June 2019 / Accepted: 4 June 2019 / Published: 6 June 2019
(This article belongs to the Special Issue Oligodendrocyte Physiology and Pathology Function)
Williams-Beuren syndrome (WBS) is caused by microdeletions of 28 genes and is characterized by cognitive disorder and hypotrophic corpus callosum (CC). Nsun5 gene, which encodes cytosine-5 RNA methyltransferase, is located in the deletion loci of WBS. We have reported that single-gene knockout of Nsun5 (Nsun5-KO) in mice impairs spatial cognition. Herein, we report that postnatal day (PND) 60 Nsun5-KO mice showed the volumetric reduction of CC with a decline in the number of myelinated axons and loose myelin sheath. Nsun5 was highly expressed in callosal oligodendrocyte precursor cells (OPCs) and oligodendrocytes (OLs) from PND7 to PND28. The numbers of OPCs and OLs in CC of PND7-28 Nsun5-KO mice were significantly reduced compared to wild-type littermates. Immunohistochemistry and Western blot analyses of myelin basic protein (MBP) showed the hypomyelination in the CC of PND28 Nsun5-KO mice. The Nsun5 deletion suppressed the proliferation of OPCs but did not affect transition of radial glial cells into OPCs or cell cycle exit of OPCs. The protein levels, rather than transcriptional levels, of CDK1, CDK2 and Cdc42 in the CC of PND7 and PND14 Nsun5-KO mice were reduced. These findings point to the involvement of Nsun5 deletion in agenesis of CC observed in WBS. View Full-Text
Keywords: Nsun5; Williams-Beuren syndrome (WBS); corpus callosum (CC); oligodendrocyte (OL); myelination Nsun5; Williams-Beuren syndrome (WBS); corpus callosum (CC); oligodendrocyte (OL); myelination
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Yuan, Z.; Chen, P.; Zhang, T.; Shen, B.; Chen, L. Agenesis and Hypomyelination of Corpus Callosum in Mice Lacking Nsun5, an RNA Methyltransferase. Cells 2019, 8, 552.

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