[HTML][HTML] Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase
C Li, P Chen, A Palladino, S Narayan… - Journal of Biological …, 2010 - ASBMB
The mechanism of insulin dysregulation in children with hyperinsulinism associated with
inactivating mutations of short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD) was
examined in mice with a knock-out of the hadh gene (hadh−/−). The hadh−/− mice had
reduced levels of plasma glucose and elevated plasma insulin levels, similar to children with
SCHAD deficiency. hadh−/− mice were hypersensitive to oral amino acid with decrease of
glucose level and elevation of insulin. Hypersensitivity to oral amino acid in hadh−/− mice …
inactivating mutations of short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD) was
examined in mice with a knock-out of the hadh gene (hadh−/−). The hadh−/− mice had
reduced levels of plasma glucose and elevated plasma insulin levels, similar to children with
SCHAD deficiency. hadh−/− mice were hypersensitive to oral amino acid with decrease of
glucose level and elevation of insulin. Hypersensitivity to oral amino acid in hadh−/− mice …