Lipids of liver, kidney, spleen and muscle in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation

The lipid compositions of liver, kidney, spleen and muscle in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation were analyzed and compared with age-matched controls. The contents of cholesterol and phospholipids (μmol/g) in each organ were within the normal range. Diminished proportions of unsaturated fatty acids were observed in ethanolamine phosphoglycerides (EPG) of the liver. The proportion of linoleic acid decreased to less than half of the normal level in EPG of liver, kidney and spleen.