Establishment of clonal myogenic cell lines from severely affected dystrophic muscles - CDK4 maintains the myogenic population
| العنوان: | Establishment of clonal myogenic cell lines from severely affected dystrophic muscles - CDK4 maintains the myogenic population |
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| المؤلفون: | Kathryn R. Wagner, Jennifer C. J. Chen, Jerry W. Shay, Guido Stadler, Jérôme D. Robin, Charles P. Emerson, Woodring E. Wright |
| المصدر: | Skeletal Muscle, Vol 1, Iss 1, p 12 (2011) Skeletal Muscle |
| بيانات النشر: | Springer Science and Business Media LLC, 2011. |
| سنة النشر: | 2011 |
| مصطلحات موضوعية: | Cell type, Telomerase, lcsh:Diseases of the musculoskeletal system, Population, Biology, 03 medical and health sciences, 0302 clinical medicine, medicine, Myocyte, Facioscapulohumeral muscular dystrophy, Orthopedics and Sports Medicine, Telomerase reverse transcriptase, Myopathy, education, Molecular Biology, 030304 developmental biology, Genetics, 0303 health sciences, education.field_of_study, Research, Cell Biology, medicine.disease, Telomere, Cell biology, lcsh:RC925-935, medicine.symptom, 030217 neurology & neurosurgery |
| الوصف: | Background A hallmark of muscular dystrophies is the replacement of muscle by connective tissue. Muscle biopsies from patients severely affected with facioscapulohumeral muscular dystrophy (FSHD) may contain few myogenic cells. Because the chromosomal contraction at 4q35 linked to FSHD is thought to cause a defect within myogenic cells, it is important to study this particular cell type, rather than the fibroblasts and adipocytes of the endomysial fibrosis, to understand the mechanism leading to myopathy. Results We present a protocol to establish clonal myogenic cell lines from even severely dystrophic muscle that has been replaced mostly by fat, using overexpression of CDK4 and the catalytic component of telomerase (human telomerase reverse transcriptase; hTERT), and a subsequent cloning step. hTERT is necessary to compensate for telomere loss during in vitro cultivation, while CDK4 prevents a telomere-independent growth arrest affecting CD56+ myogenic cells, but not their CD56- counterpart, in vitro. Conclusions These immortal cell lines are valuable tools to reproducibly study the effect of the FSHD mutation within myoblasts isolated from muscles that have been severely affected by the disease, without the confounding influence of variable amounts of contaminating connective-tissue cells. |
| تدمد: | 2044-5040 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::32af159451b8735ba5533f52b795e2ccTest https://doi.org/10.1186/2044-5040-1-12Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....32af159451b8735ba5533f52b795e2cc |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 20445040 |
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