Generation of GZKHQi001-A and GZWWTi001-A, two induced pluripotent stem cell lines derived from peripheral blood mononuclear cells of Duchenne muscular dystrophy patients
Autor: | Sun Xiaofang, Xue Yanting, Song Bing, Chen Yuchang, Xie Yuhuan, Ouyang Shuming, Xian Yexing, Li Shaoying, Xiong Zeyu, Xie Yingjun, Li Haoxian |
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Rok vydání: | 2017 |
Předmět: |
musculoskeletal diseases
0301 basic medicine Male congenital hereditary and neonatal diseases and abnormalities Duchenne muscular dystrophy Induced Pluripotent Stem Cells Cardiomyopathy Cell Culture Techniques Mice SCID Biology Cell Line 03 medical and health sciences Mice Inbred NOD medicine Myocyte Animals Humans Muscular dystrophy Induced pluripotent stem cell lcsh:QH301-705.5 Muscle contracture Reproducibility of Results Cell Biology General Medicine medicine.disease Muscle atrophy nervous system diseases Muscular Dystrophy Duchenne 030104 developmental biology lcsh:Biology (General) Child Preschool Cancer research biology.protein Leukocytes Mononuclear medicine.symptom Dystrophin Developmental Biology Microsatellite Repeats |
Zdroj: | Stem Cell Research, Vol 28, Iss, Pp 25-28 (2018) |
ISSN: | 1876-7753 |
Popis: | Duchenne muscular dystrophy (DMD) is an X-linked disease caused by mutations in the DMD gene, which spans ~2.4 Mb 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. |
Databáze: | OpenAIRE |
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