國家衛生研究院 NHRI:Item 3990099045/10676

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Please use this identifier to cite or link to this item: http://ir.nhri.org.tw/handle/3990099045/10676

Title:	Utrophin compensates dystrophin loss during mouse spermatogenesis
Authors:	Chen, HC;Chin, YF;Lundy, DJ;Liang, CT;Chi, YH;Kuo, P;Hsieh, PCH
Contributors:	Institute of Biotechnology and Pharmaceutical Research
Abstract:	Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder resulting from mutations in the dystrophin gene. The mdx/utrn -/- mouse, lacking in both dystrophin and its autosomal homologue utrophin, is commonly used to model the clinical symptoms of DMD. Interestingly, these mice are infertile but the mechanisms underlying this phenomenon remain unclear. Using dystrophin deficient mdx mouse and utrophin haplodeficient mdx/utrn +/- mouse models, we demonstrate the contribution of Dp427 (full-length dystrophin) and utrophin to testis and epididymis development, as well as spermatogenesis. We show that Dp427 deficiency disturbed the balance between proliferation and apoptosis of germ cells during spermatogenesis, which was further disrupted with utrophin haplodeficiency, deciphering a compensatory role of utrophin for dystrophin in the male reproductive system. In the spermatozoa, we have found a compensatory response of utrophin to dystrophin deficiency - namely the upregulation and relocation of utrophin to the flagellar midpiece. This study demonstrates the contribution of Dp427 and utrophin in male fertility, suggesting a potential pathology in DMD patients.
Date:	2017-08-07
Relation:	Scientific Reports. 2017 Aug 7;7:Article number 7372.
Link to:	http://dx.doi.org/10.1038/s41598-017-05993-8
JIF/Ranking 2023:	http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=NHRI&SrcApp=NHRI_IR&KeyISSN=2045-2322&DestApp=IC2JCR
Cited Times(WOS):	https://www.webofscience.com/wos/woscc/full-record/WOS:000407080100002
Cited Times(Scopus):	http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=85027255091
Appears in Collections:	[Ya-Hui Chi] Periodical Articles

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