Leukocyte docosahexaenoic acid in juvenile form of ceroid-lipofuscinosis.
Leukocyte glycerolipid fatty acid compositions were determined in four patients of juvenile form of neuronal ceroid-lipofuscinosis (Battens disease), their parents and age-matched controls. A 54 to 93% reduction in docosahexaenoic acid (22:6, n-3) was noted in patient leukocytes. The parents also showed reduction in docosahexaenoic acid content, but to a lesser degree. The levels of linolenic family (n-6) polyunsaturated fatty acids were unchanged suggesting that the metabolic defect in this disease is specific to the linolenic family (n-3) of fatty acids.
D000293:Adolescent; D002648:Child; D005231:Fatty Acids, Unsaturated / Q000097:blood*; D005260:Female; D005989:Glycerides / Q000097:blood; D006801:Humans; D007962:Leukocytes / Q000378:metabolism*; D008064:Lipidoses / Q000097:blood*; D008297:Male
D005231:Fatty Acids, Unsaturated; D005989:Glycerides