Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose–fatty acid cycle?
2003 ◽
Vol 31
(6)
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pp. 1137-1139
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Keyword(s):
Β Cells
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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.
2013 ◽
Vol 98
(2)
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pp. 496-501
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2000 ◽
Vol 23
(1)
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pp. 27-44
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2016 ◽
Vol 291
(19)
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pp. 10162-10172
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1996 ◽
Vol 39
(1)
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pp. 185-188
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Keyword(s):
1994 ◽
Vol 153
(10)
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pp. 745-750
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2007 ◽
Vol 91
(2)
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pp. 205-206
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2019 ◽
Vol 152
(Supplement_1)
◽
pp. S9-S9