4418 L-Isoleucine L-Isoleucine is one of the essential amino acids that cannot be made by the body and is known for its ability to help endurance and assist in the repair and rebuilding of muscle. This amino acid is important to body builders as it helps boost energy and helps the body recover from training. L-Isoleucine is also classified as a branched-chain amino acid (BCAA). It helps promote muscle recovery after exercise. Isoleucine is actually broken down for energy within the muscle tissue. It is important in hemoglobin synthesis and regulation of blood sugar and energy levels. 73-32-5 6306 C6H13NO2 131.17 White powder. 285.5 dec°C 3.44E+004 mg/L (at 25°C) Absorbed from the small intestine by a sodium-dependent active-transport process (Applies to Valine, Leucine and Isoleucine) <br/>This group of essential amino acids are identified as the branched-chain amino acids, BCAAs. Because this arrangement of carbon atoms cannot be made by humans, these amino acids are an essential element in the diet. The catabolism of all three compounds initiates in muscle and yields NADH and FADH2 which can be utilized for ATP generation. The catabolism of all three of these amino acids uses the same enzymes in the first two steps. The first step in each case is a transamination using a single BCAA aminotransferase, with a-ketoglutarate as amine acceptor. As a result, three different a-keto acids are produced and are oxidized using a common branched-chain a-keto acid dehydrogenase, yielding the three different CoA derivatives. Subsequently the metabolic pathways diverge, producing many intermediates. <br/>The principal product from valine is propionylCoA, the glucogenic precursor of succinyl-CoA. Isoleucine catabolism terminates with production of acetylCoA and propionylCoA; thus isoleucine is both glucogenic and ketogenic. Leucine gives rise to acetylCoA and acetoacetylCoA, and is thus classified as strictly ketogenic. <br/>There are a number of genetic diseases associated with faulty catabolism of the BCAAs. The most common defect is in the branched-chain a-keto acid dehydrogenase. Since there is only one dehydrogenase enzyme for all three amino acids, all three a-keto acids accumulate and are excreted in the urine. The disease is known as Maple syrup urine disease because of the characteristic odor of the urine in afflicted individuals. Mental retardation in these cases is extensive. Unfortunately, since these are essential amino acids, they cannot be heavily restricted in the diet; ultimately, the life of afflicted individuals is short and development is abnormal The main neurological problems are due to poor formation of myelin in the CNS. Hepatic No indication of carcinogenicity to humans (not listed by IARC). The branched-chain amino acids may have antihepatic encephalopathy activity in some. They may also have anticatabolic and antitardive dyskinesia activity. Symptoms of hypoglycemia 2014-08-29T06:35:06Z 2026-04-17T18:55:30Z Ile C00407 17191 ILE DB00167 ILE true CC[C@H](C)[C@H](N)C(O)=O C6H13NO2 InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1 AGPKZVBTJJNPAG-WHFBIAKZSA-N 131.1729 131.094628665 Endogenous Solid -1.7 HMDB00172 CHEMBL1233584 6067 <p>Nelli I. Zhdanova, Tatyana V. Leonova, Ljudmila F. Kozyreva, &#8220;Method of producing L-isoleucine using Brevibacterium flavum.&#8221; U.S. Patent US4237228, issued February, 1962.</p> CHEM003324 DTXSID1047441 NS00009745 (2S,3S)-2-amino-3-methylpentanoic acid