Abstract
Abstract Liver biopsies from two patients with glucose-6-phosphatase deficiency were found to contain a high de novo fatty acid synthesizing activity from citrate as the carbon source. Liver biopsies from controls, from patients with debranching enzyme deficiency or from one patient with insulinoma showed much lower rates of long-chain fatty acid synthesis. From this investigation it is concluded that there is no correlation between fatty acid synthesis in the liver and the insulin and/or glucose levels in the blood. However, there may be a positive correlation between the activity of the rate-limiting step in long-chain fatty acid synthesis and the lactate level in the blood.
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