Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose–fatty acid cycle?
S Eaton, I Chatziandreou, S Krywawych… - Biochemical Society …, 2003 - portlandpress.com
Biochemical Society Transactions, 2003•portlandpress.com
Hyperinsulinism of infancy is caused by inappropriate insulin secretion in pancreatic β-cells,
even when blood glucose is low. Several molecular defects are known to cause
hyperinsulinism of infancy, such as KATP channelopathies and regulatory defects of
glucokinase and glutamate dehydrogenase. Although defects of fatty acid oxidation have not
previously been known to cause hyperinsulinism, patients with deficiency in SCHAD (short-
chain 3-hydroxyacyl-CoA dehydrogenase; an enzyme of mitochondrial β-oxidation) have …
even when blood glucose is low. Several molecular defects are known to cause
hyperinsulinism of infancy, such as KATP channelopathies and regulatory defects of
glucokinase and glutamate dehydrogenase. Although defects of fatty acid oxidation have not
previously been known to cause hyperinsulinism, patients with deficiency in SCHAD (short-
chain 3-hydroxyacyl-CoA dehydrogenase; an enzyme of mitochondrial β-oxidation) have …
Hyperinsulinism of infancy is caused by inappropriate insulin secretion in pancreatic β-cells, even when blood glucose is low. Several molecular defects are known to cause hyperinsulinism of infancy, such as KATP channelopathies and regulatory defects of glucokinase and glutamate dehydrogenase. Although defects of fatty acid oxidation have not previously been known to cause hyperinsulinism, patients with deficiency in SCHAD (short-chain 3-hydroxyacyl-CoA dehydrogenase; an enzyme of mitochondrial β-oxidation) have hyperinsulinism. A novel link between fatty acid oxidation and insulin secretion may explain hyperinsulinism in these patients.
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