New indications and controversies in arginine therapy.
Arginine is an important, versatile and a conditionally essential amino acid. Besides serving as a building block for tissue proteins, arginine plays a critical role in ammonia detoxification, and nitric oxide and creatine production. Arginine supplementation is an essential component for the treatment of urea cycle defects but recently some reservations have been raised with regards to the doses used in the treatment regimens of these disorders. In recent years, arginine supplementation or restriction has been proposed and trialled in several disorders, including vascular diseases and asthma, mitochondrial encephalopathy lactic acidosis and stroke-like episodes (MELAS), glutaric aciduria type I and disorders of creatine metabolism, both production and transportation into the central nervous system. Herein we present new therapeutic indications and controversies surrounding arginine supplementation or deprivation.
Coman
David
D
Metabolic Service, Genetic Health Services Victoria, Murdoch Children's Research Institute, 10th Floor, The Royal Children's Hospital, Flemington Road, Parkville 3052, Melbourne, Australia.
Yaplito-Lee
Joy
J
Boneh
Avihu
A
D000140:Acidosis, Lactic / Q000188:drug therapy / Q000378:metabolism; D000592:Amino Acid Metabolism, Inborn Errors / Q000188:drug therapy / Q000378:metabolism; D001120:Arginine / Q000502:physiology* / Q000627:therapeutic use*; D002490:Central Nervous System / Q000378:metabolism*; D003401:Creatine / Q000096:biosynthesis; D019587:Dietary Supplements; D006801:Humans; D017241:MELAS Syndrome / Q000188:drug therapy / Q000378:metabolism; D017240:Mitochondrial Myopathies / Q000188:drug therapy / Q000378:metabolism; D009569:Nitric Oxide / Q000096:biosynthesis; D014652:Vascular Diseases / Q000188:drug therapy / Q000378:metabolism
D009569:Nitric Oxide; D001120:Arginine; D003401:Creatine