دورية أكاديمية
McArdle disease does not affect skeletal muscle fibre type profiles in humans
العنوان: | McArdle disease does not affect skeletal muscle fibre type profiles in humans |
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المؤلفون: | Tertius Abraham Kohn, Timothy David Noakes, Dale Elizabeth Rae, Juan Carlos Rubio, Alfredo Santalla, Gisela Nogales-Gadea, Tomas Pinós, Miguel A. Martín, Joaquin Arenas, Alejandro Lucia |
المصدر: | Biology Open, Vol 3, Iss 12, Pp 1224-1227 (2014) |
بيانات النشر: | The Company of Biologists, 2014. |
سنة النشر: | 2014 |
المجموعة: | LCC:Science LCC:Biology (General) |
مصطلحات موضوعية: | Myosin heavy chain, Glycogen storage disease V, Phosphorylase deficiency, Science, Biology (General), QH301-705.5 |
الوصف: | Patients suffering from glycogen storage disease V (McArdle disease) were shown to have higher surface electrical activity in their skeletal muscles when exercising at the same intensity as their healthy counterparts, indicating more muscle fibre recruitment. To explain this phenomenon, this study investigated whether muscle fibre type is shifted towards a predominance in type I fibres as a consequence of the disease. Muscle biopsies from the Biceps brachii (BB) (n = 9) or Vastus lateralis (VL) (n = 8) were collected over a 13-year period from male and female patients diagnosed with McArdle disease, analysed for myosin heavy chain (MHC) isoform content using SDS-PAGE, and compared to healthy controls (BB: n = 3; VL: n = 10). All three isoforms were expressed and no difference in isoform expression in VL was found between the McArdle patients and healthy controls (MHC I: 33±19% vs. 43±7%; MHC IIa: 52±9% vs. 40±7%; MHC IIx: 15±18% vs. 17±9%). Similarly, the BB isoform content was also not different between the two groups (MHC I: 33±14% vs. 30±11%; MHC IIa: 46±17% vs. 39±5%; MHC IIx: 21±13% vs. 31±14%). In conclusion, fibre type distribution does not seem to explain the higher surface EMG in McArdle patients. Future studies need to investigate muscle fibre size and contractility of McArdle patients. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 2046-6390 |
العلاقة: | http://bio.biologists.org/content/3/12/1224Test; https://doaj.org/toc/2046-6390Test |
DOI: | 10.1242/bio.20149548 |
الوصول الحر: | https://doaj.org/article/07c1ce30caa147ad9fbaa4f6b508a307Test |
رقم الانضمام: | edsdoj.07c1ce30caa147ad9fbaa4f6b508a307 |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 20466390 |
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DOI: | 10.1242/bio.20149548 |