4383 T3D4329 Ornithine Ornithine is an amino acid produced in the urea cycle by the splitting off of urea from arginine. It is a central part of the urea cycle, which allows for the disposal of excess nitrogen. L-Ornithine is also a precursor of citrulline and arginine. In order for ornithine produced in the cytosol to be converted to citrulline, it must first cross the inner mitochondrial membrane into the mitochondrial matrix where it is carbamylated by ornithine transcarbamylase. This transfer is mediated by the mitochondrial ornithine transporter (SLC25A15; AF112968; ORNT1). Mutations in the mitochondrial ornithine transporter result in hyperammonemia, hyperornithinemia, homocitrullinuria (HHH) syndrome, a disorder of the urea cycle. The pathophysiology of thef the disease may involve diminished ornithine transport into mitochondria, resulting in ornithine accumulation in the cytoplasm and reduced ability to clear carbamoyl phosphate and ammonia loads. (OMIM 838970). (A3420). 70-26-8 6262 C5H12N2O2 White powder. 140°C Appreciable Absorbed from the small intestine via a sodium-dependent active transport process L-Ornithine is metabolised to L-arginine. L-arginine stimulates the pituitary release of growth hormone. Burns or other injuries affect the state of L-arginine in tissues throughout the body. As De novo synthesis of L-arginine during these conditions is usually not sufficient for normal immune function, nor for normal protein synthesis, L-ornithine may have immunomodulatory and wound-healing activities under these conditions (by virtue of its metabolism to L-arginine). Ornithine undergoes extensive metabolism in the liver to L-arginine, polyamines, and proline, and several other metabolites. Oral, rat LD<sub>50</sub> = 10000 mg/kg No indication of carcinogenicity to humans (not listed by IARC). Used for nutritional supplementation, also for treating dietary shortage or imbalance. It has been claimed that ornithine improves athletic performance, has anabolic effects, has wound-healing effects, and is immuno-enhancing. Chronically high levels of ornithine are associated with at least 9 inborn errors of metabolism including: Cystathionine Beta-Synthase Deficiency, Hyperornithinemia with gyrate atrophy, Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, Hyperprolinemia Type II, Lysinuric Protein Intolerance, Ornithine Aminotransferase Deficiency, Ornithine Transcarbamylase Deficiency and Prolinemia Type II. 2014-08-29T06:27:54Z 2026-05-14T16:24:09Z Ornithine C01602 15729 L-ORNITHINE DB00129 ORN true NCCC[C@H](N)C(O)=O C5H12N2O2 InChI=1S/C5H12N2O2/c6-3-1-2-4(7)5(8)9/h4H,1-3,6-7H2,(H,8,9)/t4-/m0/s1 AHLPHDHHMVZTML-BYPYZUCNSA-N 132.161 132.089877638 Endogenous Solid -4.22 HMDB00214 CHEMBL446143 6026 <p>Takayasu Tsuchida, Haruo Uchibori, Yoshitaka Nishimoto, &#8220;Process and microorganism for producing L-ornithine by Corynebacterium, Brevibacterium, or Athrobacter.&#8221; U.S. Patent US5188947, issued January, 1970.</p> CHEM003289 DTXSID00860383 NS00013927 (2S)-2,5-diaminopentanoic acid 89.34 33.20850000000001 13.85326140764592 4 4 3 2.666422703275891 10.2868213384358 1 0 -3.64 0.11 1.72e+02 g/l