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Article

Mitochondrial GTP insensitivity contributes to hypoglycemia in hyperinsulinemia hyperammonemia by inhibiting glucagon release.

Diabetes - 1 Dec 2014

Kibbey Richard G, Choi Cheol Soo, Lee Hui-Young, Cabrera Over, Pongratz Rebecca L, Zhao Xiaojian, Birkenfeld Andreas L, Li Changhong, Berggren Per-Olof, Stanley Charles, Shulman Gerald I

Abstract excerpt

Mitochondrial GTP (mtGTP)-insensitive mutations in glutamate dehydrogenase (GDH(H454Y)) result in fasting and amino acid-induced hypoglycemia in hyperinsulinemia hyperammonemia (HI/HA). Surprisingly, hypoglycemia may occur in this disorder despite appropriately suppressed insulin. To better understand the islet-specific contribution, transgenic mice expressing the human activating mutation in β-cells (H454Y mice)...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 25024374
DOI: 10.2337/db14-0783
PMCID: PMC4237996

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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