title: Effect of dietary supplementation with branched-chain amino acids on spontaneous motor activity and muscle function in beta,beta'-iminodipropionitrile-treated rats: a model for motorneuropathy.
abstract: Beta,beta'-iminodipropionitrile (IDPN)-induced muscle weakness in rats is a model for motorneuropathy diseases. The effects of oral administration of branched-chain amino acids on the progression of muscle weakness and muscle atrophy induced by the administration of IDPN in the drinking water, were investigated in this study. The spontaneous motor activity of the animals, as measured with a running wheel, slowly declined after IDPN administration, reaching a steady and low level at approximately week 15. Progressive muscle weakness and muscle atrophy were observed beginning at approximately 15 weeks after the initiation of administration of IDPN. Administration of branched-chain amino acids (BCAA) as a dietary supplement did not improve the lowered spontaneous motor activity seen in IDPN animals, but it did significantly improve measures of postural weakness and muscle strength [range of F values (2, 24) = 4.1-9.5, p < 0.05]. Plasma creatinine of the IDPN-treated rats was markedly elevated, and BCAA administration also significantly suppressed this elevation [F(2, 24) = 41.2, p < 0.05]. Moreover, although BCAA in plasma were elevated in the rats administered BCAA [range of F values (2, 21) = 25.7-29.7, p < 0.05], skeletal muscle showed no differences (at the p < 0.05 level) in the amounts of BCAA, whether or not BCAA were administered. These data suggest that the BCAA taken up in the skeletal muscle were utilized in the muscle of motorneuropathic rats and improved muscle function, such as energy metabolism, and that the BCAA treatment is one important therapeutic approach for retarding the progression of muscle weakness seen in certain neuromuscular disorders.
authors.affiliation: Central Research Laboratories, Ajinomoto Co., Inc., Kawasaki, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Kitahara
authors.forename: T
authors.initials: T
authors.lastname: Maki
authors.forename: K
authors.initials: K
authors.lastname: Torii
mesh_terms: D000597:Amino Acids, Branched-Chain / Q000494:pharmacology*; D000818:Animals; D004195:Disease Models, Animal; D005527:Food, Fortified; D008297:Male; D009043:Motor Activity / Q000187:drug effects*; D016472:Motor Neuron Disease / Q000139:chemically induced* / Q000473:pathology; D018482:Muscle, Skeletal / Q000187:drug effects; D009132:Muscles / Q000187:drug effects*; D009498:Neurotoxins / Q000494:pharmacology*; D009570:Nitriles / Q000494:pharmacology*; D051381:Rats; D017207:Rats, Sprague-Dawley; D013116:Spinal Cord / Q000187:drug effects
chemical_list: D000597:Amino Acids, Branched-Chain; D009498:Neurotoxins; D009570:Nitriles; C008110:3,3'-iminodipropionitrile