“…Conjugation of these amino acids may contribute significantly to the clinical symptoms of isovaleric acidemia, not only because of the conjugation products themselves being potentially toxic, but also because of a depletion of these amino acids owing to excretion of the conjugation products, in combination to dietary protein Table 2 Amino-acid concentrations in the urine and serum of treated isovaleric acidemia patients restriction. Deficiencies in the above-mentioned free amino acids are seen to be associated with the following clinical symptoms: retarded growth, eating disorders, decreased immunity, neurological disorders, thrombocytopenia, neutropenia, pancytopenia, cyanosis, listlessness and lethargy (Cohn et al, 1977;Huether, 1986;Contamira et al, 1991;van der Schot et al, 1994;Mero, 1999;Balch and Balch, 2000;Bassit et al, 2002). These clinical symptoms are identical to those associated with isovaleric acidemia.…”