Adipose fatty acid composition in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation.

The fatty acid composition of the triglyceride in adipose tissue in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation was analyzed and compared with age-matched controls. The proportions of linoleic acid, linolenic acid and arachidonic acid were decreased to less than half of normal level. There was a decrease in total unsaturated fatty acids and an increase in palmitic acid. These results revealed an undeveloped pattern of fatty acid composition in adipose tissue in the patient.