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The organic compound citrulline is an α-amino acid. [2] Its name is derived from citrullus, the Latin word for watermelon.Although named and described by gastroenterologists since the late 19th century, it was first isolated from watermelon in 1914 by Japanese researchers Yatarō Koga (古賀彌太郎) and Ryō Ōtake (大嶽了) [3] [4] and further codified by Mitsunori Wada of Tokyo ...
In enzymology, a citrullinase (EC 3.5.1.20) is an enzyme that catalyzes the chemical reaction. L-citrulline + H 2 O L-ornithine + CO 2 + NH 3. Thus, the two substrates of this enzyme are L-citrulline and H 2 O, whereas its 3 products are L-ornithine, CO 2, and NH 3.
Citrulline is not one of the 20 standard amino acids encoded by DNA in the genetic code. Instead, it is the result of a post-translational modification. Citrullination is distinct from the formation of the free amino acid citrulline as part of the urea cycle or as a byproduct of enzymes of the nitric oxide synthase family.
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L-Homocitrulline is an amino acid and can be detected in larger amounts in the urine of individuals with urea cycle disorders. At present, it is thought that the depletion of the ornithine supply causes the accumulation of carbamyl-phosphate in the urea cycle which may be responsible for the enhanced synthesis of homocitrulline and homoarginine .
Some cells synthesize argininosuccinic acid from citrulline and aspartic acid and use it as a precursor for arginine in the urea cycle (or citrulline-NO cycle), releasing fumarate as a by-product to enter the TCA cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase. [3] [4]
Citrullinemia is an autosomal recessive urea cycle disorder that causes ammonia and other toxic substances to accumulate in the blood. [1]Two forms of citrullinemia have been described, both having different signs and symptoms, and are caused by mutations in different genes.
Beneficial side effects are less common; some examples, in many cases of side-effects that ultimately gained regulatory approval as intended effects, are: Bevacizumab ( Avastin ), used to slow the growth of blood vessels, has been used against dry age-related macular degeneration , as well as macular edema from diseases such as diabetic ...
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