Results 51 to 60 of about 105,036 (166)

A " NEW " DISORDER OF ISOLEUCINE CATABOLISM

The Lancet, 1971
A disorder of isoleucine catabolism was found in a child with intermittent metabolic acidosis. The block occurs at the stage of propionate synthesis, and two intermediates--alpha-methylacetoacetate and alpha-methyl-beta-hydroxybutyrate--accumulate. The condition is apparently inherited.
R S, Daum, P H, Lamm, O A, Mamer, C R, Scriver   +3 more
openaire   +3 more sources

Atypical Presentation of IARS1‐Related Disorder: Expanding the Phenotype and Genotype

JIMD Reports
Aminoacyl‐tRNA synthetases (ARSs) catalyze the formation of aminoacyl‐tRNA, which is required for protein translation. A growing number of cases are associated with ARS deficiencies.
Parith Wongkittichote, Kira E. Jonatzke, Benjamin T. Hyde, Lance W. Peterson, Mai He, Robert C. McKinstry, Anthony Antonellis, Marwan Shinawi   +7 more
doaj   +1 more source

Protein and amino acid profile of Ruditapes decussatus and Ruditapes philippinarum from Çanakkale Strait, Türkiye

Oceanological and Hydrobiological Studies
Protein content was lowest in April 2018 and highest in May 2018 for Ruditapes decussatus (Linnaeus 1758), and was lowest in September 2017 and highest in December 2017 for Ruditapes philippinarum (Adams, Reeve 1850).
Bilgen Pervin Vural, Acarlı Sefa, Berber Selçuk, Celik Pinar, Kale Semih   +4 more
doaj   +1 more source

Synthesis of Isoleucine

Journal of the agricultural chemical society of Japan, 1962
不飽和カルボン酸に対するグリニヤール試薬の1, 4付加反応をイソロイシンの合成に応用し,新合成法を確立した.すなわち,クロトン酸およびそのsec-ブチルエステルにエチルマグネシウムブロミドを作用せしめた後,その反応コンプレックスにハロゲンを付加し,得られたα-ハロゲンカルボン酸をアミノ化してイソロイシンを合成した.なお,この際グリニヤールコンプレックスへのヨウ素付加においてスレオ型が優先して生ずることより,その付加が隣接基の立体効果をうける現象を認めた.
openaire   +2 more sources

Acrodermatitis dysmetabolica as a sign of methylmalonic aciduria decompensation

Clinical Case Reports, 2018
Key Clinical Message Methylmalonic aciduria children must follow an adequate diet with low protein intake and should be regularly monitored to prevent complications.
Joana Rosa, Ana Beatriz Fraga, Rita deCarvalho, Ana Lúcia Maia, Ana Luísa Rodrigues, Maria Fernanda Gomes   +5 more
doaj   +1 more source

Effect of Intravenous L-Isoleucine Infusion upon Concentration of Free Isoleucine in Milk

Journal of Dairy Science, 1976
The growth of Streptococcus agalactiae in milk is inhibited by elevated free isoleucine. Free amino acid concentrations in both plasma and milk from three lactating dairy cows were studied after intravenous infusions of L-isoleucine at 62, 300, 380, and 840 mumol/kg of body weight. Free isoleucine was lower in milk than in plasma.
O, Popescu, M J, Allison, R W, Brown
openaire   +2 more sources

Redetermined crystal structure of N-(β-carboxyethyl)-α-isoleucine

Acta Crystallographica Section E: Crystallographic Communications, 2015
Redetermination of the crystal structure of N-(β-carboxyethyl)-α-isoleucine, C9H18N2O3, reported earlier by Nehls et al. [Acta Cryst. (2013), E69, o172–o173], was undertaken in which the ionization state assigned to the molecule as unionized has been ...
M. Chandrarekha, N. Srinivasan, R. V. Krishnakumar   +2 more
doaj   +1 more source

THREONINE, ISOLEUCINE, AND ISOLEUCINE-VALINE MUTANTS OF SALMONELLA TYPHIMURIUM [PDF]

Genetics, 1960
E V, Glanville, M, Demerec
openaire   +2 more sources

The Effect of Dexamethasone-Mediated Atrophy on Mitochondrial Function and BCAA Metabolism During Insulin Resistance in C2C12 Myotubes

Metabolites
Background: Muscle loss during sarcopenia and atrophy is also commonly associated with age-related insulin resistance. Interestingly, branched-chain amino acids (BCAA) which are known for stimulating muscle protein synthesis are commonly elevated during ...
Kayla J. Ragland, Kipton B. Travis, Emmalie R. Spry, Toheed Zaman, Pamela M. Lundin, Roger A. Vaughan   +5 more
doaj   +1 more source

Systemic energy metabolism changes in patients with heart failure receiving specific oral nutritional supplements in combination with Mediterranean diet

Frontiers in Nutrition
BackgroundHeart failure (HF) is associated with disease-related malnutrition, all of which contribute to impaired functional capacity and adverse outcomes.
Aura D. Herrera-Martínez, Aura D. Herrera-Martínez, Concepción Muñoz Jiménez, Concepción Muñoz Jiménez, José López Aguilera, José López Aguilera, Manuel Crespin, Manuel Crespin, María Ángeles Gálvez Moreno, María Ángeles Gálvez Moreno, María José Molina Puerta, María José Molina Puerta   +11 more
doaj   +1 more source