Glycogen Storage Disease > 자유게시판

Hedberg-Oldfors, Carola; Oldfors, Anders (2015). "Polyglucosan storage myopathies". Molecular Aspects of Medicine. Echaniz-Laguna A, Lornage X, Laforêt P, Orngreen MC, Edelweiss E, Brochier G, Healthy Flow Blood Bui MT, Silva-Rojas R, Healthy Flow Blood health Birck C, Healthy Flow Blood health Lannes B, Romero NB, Vissing J, Laporte J, Böhm J. A brand new Glycogen Storage Disease Attributable to a Dominant PYGM Mutation. Ann Neurol. 2020 Aug;88(2):274-282. Echaniz-Laguna, A.; Lornage, X.; Edelweiss, E.; Laforêt, P.; Eymard, B.; Vissing, J.; Laporte, J.; Böhm, J. (October 2019). "O.5A new glycogen storage disorder attributable to a dominant mutation within the glycogen myophosphorylase gene (PYGM)". Neuromuscular Disorders. 29: Healthy Flow Blood health S39. Rose MR, Howard RS, Genet SA, McMahon CJ, Whitfield A, Healthy Flow Blood Morgan-Hughes JA. A case of myopathy related to a dystrophin gene deletion and abnormal glycogen storage. Muscle Nerve. 1993 Jan;16(1):57-62. Lucia, Alejandro; Martinuzzi, Andrea; Nogales-Gadea, Gisela; Quinlivan, Ros; Reason, Stacey; International Association for Muscle Glycogen Storage Disease examine group (December 2021). "Clinical practice tips for glycogen storage illness V & VII (McArdle disease and Tarui disease) from a world study group".

The quantity of drive that the muscle creates varies -- the muscle can contract a little or lots depending on the sign that the nerve sends. All that any muscle can do is create contraction drive. A muscle is a bundle of many cells known as fibers. You possibly can think of muscle fibers as long cylinders, and in comparison with different cells in your physique, muscle fibers are fairly massive. They are from about 1 to 40 microns lengthy and 10 to 100 microns in diameter. For comparability, a strand of hair is about 100 microns in diameter, and a typical cell in your body is about 10 microns in diameter. A muscle fiber accommodates many myofibrils, that are cylinders of muscle proteins. These proteins enable a muscle cell to contract. Myofibrils comprise two types of filaments that run along the long axis of the fiber, and these filaments are arranged in hexagonal patterns. There are thick and thin filaments. Each thick filament is surrounded by six thin filaments.

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