Fatty acid composition of hepatic triglycerides in Reye's syndrome: implications for hepatic desaturase abnormalities.
Serum NEFA profiles in Reye's syndrome are reportedly unique with a disproportionate percent made up of polyunsaturated fatty acids some of which are not ordinarily found in the serum. This pattern is also reflected in the serum triglyceride composition as well. As the liver is probably the sole source of the serum triglyceride in Rye's syndrome because patients are vomiting or in coma, the fatty acid acid composition of the liver triglyceride was examined for insight regarding the lipid abnormalities in this disease. Palmitic acid (16:0) and the sum of all the saturated fatty acids in the liver triglycerides were significantly decreased whereas the sum of the monoenoic fatty acids and the products of delta 9 desaturase activity were increased in Reye's samples. When these data were compared to the fatty acid composition of the serum triglyceride from a separate cohort of Reye's and control subjects, certain inferences regarding hepatic delta 9, delta 6, delta 5, and delta 4 desaturase activities and the elongases can be drawn from the liver and serum triglyceride fatty acid profiles which are unique. Collectively, these data reflect considerable intrahepatic fatty acid desaturation and elongation activity and/or acyl transfer from lipid to lipid of various polyunsaturated fatty acids in Reye's syndrome.
Kang
E S
ES
Department of Pediatrics, University of Tennessee-Memphis 38163.
Johnson
S B
SB
Holman
R T
RT
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D044943:Fatty Acid Desaturases / Q000378:metabolism*; D005227:Fatty Acids / Q000032:analysis / Q000097:blood / Q000378:metabolism*; D005230:Fatty Acids, Nonesterified / Q000097:blood; D006801:Humans; D007223:Infant; D008099:Liver / Q000378:metabolism*; D012016:Reference Values; D012202:Reye Syndrome / Q000097:blood / Q000201:enzymology / Q000378:metabolism*; D014280:Triglycerides / Q000032:analysis / Q000097:blood / Q000378:metabolism*
D005227:Fatty Acids; D005230:Fatty Acids, Nonesterified; D014280:Triglycerides; D044943:Fatty Acid Desaturases