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The distribution of 17 carbon fatty acids in the liver of a child with propionicacidaemia
Author(s) -
Gompertz D.
Publication year - 1971
Publication title -
lipids
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.601
H-Index - 120
eISSN - 1558-9307
pISSN - 0024-4201
DOI - 10.1007/bf02531138
Subject(s) - chemistry , fatty acid , lipidology , clinical chemistry , carbon fibers , polyunsaturated fatty acid , biochemistry , double bond , organic chemistry , distribution (mathematics) , carbon number , mathematical analysis , materials science , mathematics , composite number , composite material , alkyl
A child with propionicacidaemia due to a defective propionyl CoA carboxylase activity, accumulated odd number fatty acids in his liver. Seventeen carbon saturated and monounsaturated acids both represented 2% to 3% of the total liver fatty acids. The monounsaturated acid is demonstrated to be heptadec‐9‐enoic acid. The distribution of the 17 carbon saturated and monounsaturated acids throughout the major lipid classes is described. Enzymic degradation of lecithins and triglycerides is used to establish the positional specificity of esterification of these two fatty acids. The results indicate that when the only difference between two fatty acids, present in similar concentrations, is a single double bond, highly specific esterification patterns are retained.