Creatine

The enzyme GAMT (NOTE: image states \"GATM\" which is incorrect!) (guanidinoacetate N-methyltransferase, also known as L-arginine:glycine amidinotransferase (AGAT), EC 2.1.4. more...

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1) is a mitochondrial enzyme responsible for catalyzing the first rate-limiting step of creatine biosynthesis, and is primarily expressed in the kidneys and pancreas.

The second enzyme in the pathway (GAMT, guanidinoacetate N-methyltransferase, EC:2.1.1.2) is primarily expressed in the liver and pancreas.

Genetic deficiencies in the creatine biosynthetic pathway lead to various severe neurological defects.

Creatine Monohydrate is a commonly debated performance enhancer. Many believe creatine should be banned just like any other performance enhancing substance, but due to the legal ingredients it still remains a commonly used legal substance.

Sources

In humans, approximately half of stored creatine originates from food (mainly from fresh meat and fish). Since vegetables do not contain creatine, vegetarians clearly show lower levels of muscle creatine which rise upon creatine supplementation more than meat-eaters.

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, parkinson's disease, disuse atrophy, gyrate atrophy, McArdle's disease, Huntington's disease, miscellaneous neuromuscular diseases, mitochondrial diseases, muscular dystrophy, neuroprotection, etc.).

Two studies have indicated that creatine may be beneficial for neuromuscular disorders. First, a study 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). The neuroprotective effects of creatine in the mouse model of ALS may be due either to an increased availability of energy to injured nerve cells or to a blocking of the chemical pathway which leads to cell death.

Secondly, creatine has been demonstrated to cause modest increases in strength in people with a variety of neuromuscular disorders.

Read more at Wikipedia.org