Abstract
Proteins are mobilised during exercise, fasting or dehydration from body stores and dietary sources. Out of 20 amino acids (AA), 12 are synthesised in the body from glycolysis and citric acid cycle. Glycine, gamma-aminobutyric acid act as neurotransmitters; and phenylalanine, tyrosine, tryptophan as hormone precursors, coenzymes and pigments.[1] Isovaleric acidaemia (IVA) is an autosomal recessive disorder of AA leucine metabolism wherein enzyme deficiency causes isovaleric acid accumulation and toxicity.[2]
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