McArdle Disease and Exercise Physiology
McArdle disease (glycogen storage disease Type V; MD) is a metabolic myopathy caused by a deficiency in muscle glycogen phosphorylase. Since muscle glycogen is an important fuel for muscle during exercise, this inborn error of metabolism provides a model for understanding the role of glycogen in mus...
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| Format: | Article |
| Language: | English |
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MDPI AG
2014-02-01
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| Series: | Biology |
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| Online Access: | http://www.mdpi.com/2079-7737/3/1/157 |
| _version_ | 1797719076490969088 |
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| author | Yu Kitaoka |
| author_facet | Yu Kitaoka |
| author_sort | Yu Kitaoka |
| collection | DOAJ |
| description | McArdle disease (glycogen storage disease Type V; MD) is a metabolic myopathy caused by a deficiency in muscle glycogen phosphorylase. Since muscle glycogen is an important fuel for muscle during exercise, this inborn error of metabolism provides a model for understanding the role of glycogen in muscle function and the compensatory adaptations that occur in response to impaired glycogenolysis. Patients with MD have exercise intolerance with symptoms including premature fatigue, myalgia, and/or muscle cramps. Despite this, MD patients are able to perform prolonged exercise as a result of the “second wind” phenomenon, owing to the improved delivery of extra-muscular fuels during exercise. The present review will cover what this disease can teach us about exercise physiology, and particularly focuses on the compensatory pathways for energy delivery to muscle in the absence of glycogenolysis. |
| first_indexed | 2024-03-12T08:59:31Z |
| format | Article |
| id | doaj.art-35ce40b496d546dfad9e89a9d7af2d1f |
| institution | Directory Open Access Journal |
| issn | 2079-7737 |
| language | English |
| last_indexed | 2024-03-12T08:59:31Z |
| publishDate | 2014-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biology |
| spelling | doaj.art-35ce40b496d546dfad9e89a9d7af2d1f2023-09-02T15:45:16ZengMDPI AGBiology2079-77372014-02-013115716610.3390/biology3010157biology3010157McArdle Disease and Exercise PhysiologyYu Kitaoka0Department of Sports Sciences, The University of Tokyo, Komaba 3-8-1, Meguro-ku, Tokyo 153-8902, JapanMcArdle disease (glycogen storage disease Type V; MD) is a metabolic myopathy caused by a deficiency in muscle glycogen phosphorylase. Since muscle glycogen is an important fuel for muscle during exercise, this inborn error of metabolism provides a model for understanding the role of glycogen in muscle function and the compensatory adaptations that occur in response to impaired glycogenolysis. Patients with MD have exercise intolerance with symptoms including premature fatigue, myalgia, and/or muscle cramps. Despite this, MD patients are able to perform prolonged exercise as a result of the “second wind” phenomenon, owing to the improved delivery of extra-muscular fuels during exercise. The present review will cover what this disease can teach us about exercise physiology, and particularly focuses on the compensatory pathways for energy delivery to muscle in the absence of glycogenolysis.http://www.mdpi.com/2079-7737/3/1/157McArdle diseaseskeletal musclemetabolism |
| spellingShingle | Yu Kitaoka McArdle Disease and Exercise Physiology Biology McArdle disease skeletal muscle metabolism |
| title | McArdle Disease and Exercise Physiology |
| title_full | McArdle Disease and Exercise Physiology |
| title_fullStr | McArdle Disease and Exercise Physiology |
| title_full_unstemmed | McArdle Disease and Exercise Physiology |
| title_short | McArdle Disease and Exercise Physiology |
| title_sort | mcardle disease and exercise physiology |
| topic | McArdle disease skeletal muscle metabolism |
| url | http://www.mdpi.com/2079-7737/3/1/157 |
| work_keys_str_mv | AT yukitaoka mcardlediseaseandexercisephysiology |