Disease relevance of Isoleucine

• On the mechanisms of the formation of L-alloisoleucine and the 2-hydroxy-3-methylvaleric acid stereoisomers from L-isoleucine in maple syrup urine disease patients and in normal humans [1].
• In patients with diabetes mellitus, ketotic hypoglycemia, phenylketonuria, and obligate heterozygous parents of MSUD patients, alloisoleucine was not significantly different from healthy subjects [2].
• The patient presented with psychomotor retardation, profound hypotonia and elevated plasma levels of BCAAs, but no elevation of alloisoleucine [3].
• Modest branched-chain amino acid restriction led to the disappearance of alloisoleucine and normalization of her branched chain amino acid values, while institution of a high fat diet precipitated hypoglycemia and acidosis [4].
• Isoleucine prevented liver metastasis in nude mice, which lack T cells and natural killer T cells [5].

High impact information on Isoleucine

• As judged from the fictive initial concentration, L-alloisoleucine is distributed evenly in the total body water space [6].
• When human skin fibroblast cultures were challenged with L-isoleucine, KMV accumulated at a gradually decreased rate, whereas L-alloisoleucine accumulated at a gradually accelerated rate [7].
• The relatively small difference between normal and MSUD fibroblasts in vitro as opposed to the striking differences between healthy subjects and MSUD patients in vivo are discussed with respect to the significance of physiological mechanisms participating in the formation and degradation of L-alloisoleucine in man [7].
• Branched-chain amino acid interactions in skeletal muscle: isoleucine and L-alloisoleucine [8].
• Parenteral administration of a mixture of branched-chain amino acid (BCAA) solutions is known to alter plasma levels of the BCAA (ILE, LEU, VAL), their corresponding alpha-ketoacids (KMV, KIC, KIV) and L-alloisoleucine (ALLO), a stereoisomer of ILE [8].

Chemical compound and disease context of Isoleucine

• Of the four possible stereoisomers of isoleucine, only L-alloisoleucine and L-isoleucine were found by capillary gas chromatography in the plasma of two maple syrup urine disease (MSUD) patients, one with classical and one with variant MSUD [9].
• In plasma samples from classical maple syrup urine disease patients under therapy the average (R)-OMV/(S)-OMV ratio was 0.35 and great differences in the transamination equilibria of the diastereomeric branched-chain amino acids L-isoleucine and L-alloisoleucine were demonstrated [10].

Biological context of Isoleucine

• Transamination and oxidative decarboxylation of L-isoleucine, L-alloisoleucine and related 2-oxo acids in perfused rat hind limb muscle [11].

Anatomical context of Isoleucine

• On the mechanism of L-alloisoleucine formation: studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease [7].

Associations of Isoleucine with other chemical compounds

• Metabolism of L-isoleucine, L-alloisoleucine and corresponding 2-oxo acids in rat hind limb muscle was comparatively studied under steady-state perfusion conditions [11].
• Chromatographic separation of alloisoleucine (ALE) was achieved using a Beckman 6300 Amino Acid Analyzer equipped with a 20 cm lithium High Performance column and Li-A buffer [12].

Analytical, diagnostic and therapeutic context of Isoleucine

• Furthermore, the spontaneous resolution of enantiomers upon crystallization of the other racemate, DL-allo-isoleucine, is rationalized on the basis of the aforementioned analysis of interatomic distances in the 1:1 DL-isoleucine:DL-allo-isoleucine complex [13].

References