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首页> 外文期刊>Journal of Cellular Physiology >Correction of the FSHD myoblast differentiation defect by fusion with healthy myoblasts
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Correction of the FSHD myoblast differentiation defect by fusion with healthy myoblasts

机译：修正的FSHD成肌细胞分化缺陷与健康的成肌细胞融合

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摘要

Facioscapulohumeral dystrophy (FSHD) is a neuromuscular disease with a prevalence that could reach 1 in 8,000 characterized by progressive asymmetric muscle weakness. Myoblasts isolated from FSHD muscles exhibit morphological differentiation defects and show a distinct transcription profile. These abnormalities may be linked to the muscle weakness in FSHD patients. We have tested whether fusion of FSHD myoblasts with primary myoblasts isolated from healthy individuals could correct the differentiation defects. Our results show that the number of hybrid myotubes with normal phenotype increased with the percentage of normal myoblasts initially cultured. We demonstrated that a minimum of 50% of normal nuclei is required for a phenotypic correction of the FSHD phenotype. Moreover, transcriptomic profiles of phenotypically corrected hybrid myotubes showed that the expression of deregulated genes in FSHD myotubes became almost normal. The number of deregulated pathways also decreased from 39 in FSHD myotubes to one in hybrid myotubes formed with 40% FSHD and 60% normal myoblasts. We thus propose that while phenotypical and functional correction of FSHD is feasible, it requires more than 50% of normal myoblasts, it creates limitations for cell therapy in the FSHD context. J. Cell. Physiol. 230: 62-71, 2016. (c) 2015 Wiley Periodicals, Inc.
机译：Facioscapulohumeral营养不良症(FSHD)神经肌肉疾病的患病率可能达到1 8000年的特点是进步不对称的肌肉无力。隔绝FSHD肌肉表现形态分化和显示不同的缺陷转录形象。与FSHD患者的肌肉无力。我们已经测试了是否融合FSHD成肌细胞原发性肌母细胞与健康个人可以正确的分化缺陷。与正常表型混合肌管增加与正常的成肌细胞最初的百分比讲究的。正常的表型核是必需的FSHD表现型的校正。转录组的表型修正混合肌管显示对基因的表达在FSHD肌管几乎成为了正常。途径也减少了从39 FSHD肌管在混合肌管形成FSHD为40%和60%正常肌母细胞。而表型和功能调整FSHD是可行的,它需要超过50%的正常的成肌细胞,细胞产生限制治疗FSHD上下文。230: 62 - 71, 2016。公司。

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