Creatine and Muscular Dystrophy

Bourdakos, Nikos; Wolf, Stewart

doi:10.1001/archneur.1962.00450240017003

Cited by 21 publications

(2 citation statements)

References 24 publications

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“…In DMD, increased plasma membrane fragility and subsequent leakage of cytosolic components due to dys-trophin deficiency are generally accepted to be the primary defects. The muscle concentrations of Cr, PCr, and ATP, the ATP/ADP, PCr/Cr, and PCr/ATP ratios, as well as the phosphorylation potential are significantly decreased, whereas the calculated ADP concentration and intracellular pH are increased (88,143,211,472). Conversely, serum [Cr] is increased, resulting in creatinuria, in considerably reduced tolerance toward orally administered Cr, and, very likely due to competition of Cr and GAA for reabsorption in the kidney, in elevated urinary excretion of GAA.…”

Section: A Cr Metabolism and Muscle Diseasementioning

confidence: 89%

Creatine and Creatinine Metabolism

Wyss

Kaddurah‐Daouk

2000

Physiological Reviews

2,369

2,103

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The goal of this review is to present a comprehensive survey of the many intriguing facets of creatine (Cr) and creatinine metabolism, encompassing the pathways and regulation of Cr biosynthesis and degradation, species and tissue distribution of the enzymes and metabolites involved, and of the inherent implications for physiology and human pathology. Very recently, a series of new discoveries have been made that are bound to have distinguished implications for bioenergetics, physiology, human pathology, and clinical diagnosis and that suggest that deregulation of the creatine kinase (CK) system is associated with a variety of diseases. Disturbances of the CK system have been observed in muscle, brain, cardiac, and renal diseases as well as in cancer. On the other hand, Cr and Cr analogs such as cyclocreatine were found to have antitumor, antiviral, and antidiabetic effects and to protect tissues from hypoxic, ischemic, neurodegenerative, or muscle damage. Oral Cr ingestion is used in sports as an ergogenic aid, and some data suggest that Cr and creatinine may be precursors of food mutagens and uremic toxins. These findings are discussed in depth, the interrelationships are outlined, and all is put into a broader context to provide a more detailed understanding of the biological functions of Cr and of the CK system.

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