Generation of GZKHQi001-A and GZWWTi001-A, two induced pluripotent stem cell lines derived from peripheral blood mononuclear cells of Duchenne muscular dystrophy patients

Duchenne muscular dystrophy (DMD) is an X-linked disease caused by mutations in the DMD gene, which spans ~2.4Mb of genomic sequence at locus Xp21. This mutation results in the loss of the protein dystrophin. DMD patients die in their second or third decade due to either respiratory failure or cardi...

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Bibliographic Details
Published in:Stem cell research 2018-04, Vol.28, p.25-28
Main Authors: Yuhuan, Xie, Yingjun, Xie, Yanting, Xue, Yuchang, Chen, Bing, Song, Shaoying, Li, Haoxian, Li, Yexing, Xian, Shuming, Ouyang, Zeyu, Xiong, Xiaofang, Sun
Format: Article
Language:English
Subjects:
Animals
Cell Culture Techniques - methods
Cell Line
Child, Preschool
Humans
Induced Pluripotent Stem Cells - cytology
Leukocytes, Mononuclear - metabolism
Male
Mice, Inbred NOD
Mice, SCID
Microsatellite Repeats - genetics
Muscular Dystrophy, Duchenne - blood
Muscular Dystrophy, Duchenne - pathology
Reproducibility of Results
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Summary:Duchenne muscular dystrophy (DMD) is an X-linked disease caused by mutations in the DMD gene, which spans ~2.4Mb of genomic sequence at locus Xp21. This mutation results in the loss of the protein dystrophin. DMD patients die in their second or third decade due to either respiratory failure or cardiomyopathy, as the absence of dystrophin leads to myofiber membrane fragility and necrosis, eventually resulting in muscle atrophy and contractures. Currently, there is no effective treatment for DMD, therefore induced pluripotent stem cells from DMD patients would be a powerful tool for studying disease mechanisms.
ISSN:1873-5061
1876-7753
DOI:10.1016/j.scr.2018.01.028