Biomaterial Database

Studies on requirements for amino acids in infants with disorders of amino acid metabolism. I. Effect of alanine. 1985

D G Kelts, and D Ney, and C Bay, and J M Saudubray, and W L Nyhan

Two infants with disorders of propionate metabolism were studied at 7 months of age to determine optimum levels of intake of protein and calories to meet the requirements for essential amino acid for growth in infancy, and at the same time minimize the accumulation of toxic intermediates. An effect of alanine was found that permitted growth at otherwise limiting levels of protein intake. This was not simply an effect of nonessential nitrogen as neither glycine nor glutamic acid could substitute for alanine in this protein-sparing effect. This appears to represent further evidence of the relationship between alanine and the branched-chain amino acids and of the importance of the alanine-glucose cycle in human physiology.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008764	Methylmalonic Acid	A malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.	Acid, Methylmalonic
D009751	Nutritional Requirements	The amounts of various substances in food needed by an organism to sustain healthy life.	Dietary Requirements,Nutrition Requirements,Dietary Requirement,Nutrition Requirement,Nutritional Requirement,Requirement, Dietary,Requirement, Nutrition,Requirement, Nutritional,Requirements, Dietary,Requirements, Nutrition,Requirements, Nutritional
D011422	Propionates	Derivatives of propionic acid. Included under this heading are a broad variety of acid forms, salts, esters, and amides that contain the carboxyethane structure.	Propanoate,Propanoic Acid,Propionate,Propanoates,Propanoic Acid Derivatives,Propanoic Acids,Propionic Acid Derivatives,Propionic Acids,Acid, Propanoic,Acids, Propanoic,Acids, Propionic,Derivatives, Propanoic Acid,Derivatives, Propionic Acid
D002149	Energy Intake	Total number of calories taken in daily whether ingested or by parenteral routes.	Caloric Intake,Calorie Intake,Intake, Calorie,Intake, Energy
D004044	Dietary Proteins	Proteins obtained from foods. They are the main source of the ESSENTIAL AMINO ACIDS.	Proteins, Dietary,Dietary Protein,Protein, Dietary
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000409	Alanine	A non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases IMMUNITY, and provides energy for muscle tissue, BRAIN, and the CENTRAL NERVOUS SYSTEM.	Abufène,Alanine, L-Isomer,L-Alanine,Alanine, L Isomer,L Alanine,L-Isomer Alanine
D000592	Amino Acid Metabolism, Inborn Errors	Disorders affecting amino acid metabolism. The majority of these disorders are inherited and present in the neonatal period with metabolic disturbances (e.g., ACIDOSIS) and neurologic manifestations. They are present at birth, although they may not become symptomatic until later in life.	Amino Acidopathies, Congenital,Amino Acid Metabolism Disorders, Inborn,Amino Acid Metabolism, Inborn Error,Amino Acid Metabolism, Inherited Disorders,Amino Acidopathies, Inborn,Congenital Amino Acidopathies,Inborn Errors, Amino Acid Metabolism,Inherited Errors of Amino Acid Metabolism,Amino Acidopathy, Congenital,Amino Acidopathy, Inborn,Congenital Amino Acidopathy,Inborn Amino Acidopathies,Inborn Amino Acidopathy