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Acute Insulin Responses to Leucine in Children with the Hyperinsulinism/Hyperammonemia Syndrome

Mutations of glutamate dehydrogenase cause the hyperinsulinism/hyperammonemia syndrome by desensitizing glutamate dehydrogenase to allosteric inhibition by GTP. Normal allosteric activation of glutamate dehydrogenase by leucine is thus uninhibited, leading us to propose that children with hyperinsul...

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Autori principali: KELLY, ANDREA, NG, DIVA, FERRY, ROBERT J., GRIMBERG, ADDA, KOO-MCCOY, SAMANTHA, THORNTON, PAUL S., STANLEY, CHARLES A.
Natura: Artigo
Lingua:Inglês
Pubblicazione: 2001
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3313679/
https://ncbi.nlm.nih.gov/pubmed/11502802
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