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Creatine
The enzyme GATM (guanidinoacetate N-methyltransferase, also known as L-arginine:glycine amidinotransferase (AGAT), EC 2.1.4.1), is a mitochondrial enzyme responsible for catalyzing the first rate-limiting step of creatine biosynthesis, and is primarily expressed in the kidneys. more...
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The second enzyme in the pathway (GATM, guanidinoacetate N-methyltransferase, EC:2.1.1.2) is primarily expressed in the liver.
Genetic deficiencies in the creatine biosynthetic pathway lead to various severe neurological defects.
Sources
In humans, typically half of stored creatine originates from food (mainly from meat and fish). However, endogenous synthesis of creatine in the liver is sufficient for normal activities. This is evidenced by the fact that, even though vegetables do not contain creatine, vegetarians do not suffer from creatine deficiency. Addition of creatine to the vegetarian diet has been shown to improve athletic performance . Vegetarian creatine can be obtained via chemical synthesis using plant-derived amino acids.
Creatine and the treatment of muscular diseases
Creatine supplementation has been, and continues to be, investigated as a possible therapeutic approach for the treatment of muscular, neurological and neuromuscular diseases (arthritis, congestive heart failure, disuse atrophy, gyrate atrophy, McArdle's disease, Huntington's disease, miscellaneous neuromuscular diseases, mitochondrial diseases, muscular dystrophy, neuroprotection, etc.).
Two scientific studies have indicated that creatine may be beneficial for neuromuscular disorders. First, a study (Klivenyi et al. 1999) by MDA-funded researcher M. Flint Beal of Cornell University Medical Center demonstrated that creatine was twice as effective as the prescription drug riluzole in extending the lives of mice with the degenerative neural disease amyotrophic lateral sclerosis (ALS, or Lou Gehrig's disease). Beal suspects that the neuroprotective effects of creatine in the mouse model of ALS are due either to an increased availability of energy to injured nerve cells or to a blocking of the chemical pathway that leads to cell death.
Second, a study by Canadian researchers Mark Tarnopolsky and Joan Martin of McMaster University Medical Center in Hamilton, Ontario found that creatine can cause modest increases in strength in people with a variety of neuromuscular disorders. The latter paper was published in the March 1999 issue of Neurology.
Creatine as a diagnostic of kidney function
Hospitals and doctors routinely measure blood creatinine levels to determine kidney function. Creatine is broken down to creatinine, which is eliminated through the kidneys.
History of use as a nutritional supplement
In 1912, researchers found that ingesting creatine can dramatically boost the creatine content of the muscle. In the late 1920s, after finding that the intramuscular stores of creatine can be increased by ingesting creatine in larger than normal amounts, scientists discovered creatine phosphate, and determined that creatine is a key player in the metabolism of skeletal muscle.
Read more at Wikipedia.org
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