Mitochondrial GTP Insensitivity Contributes to Hypoglycemia in Hyperinsulinemia Hyperammonemia by Inhibiting Glucagon Release
نویسندگان
چکیده
منابع مشابه
Mitochondrial GTP Insensitivity Contributes to Hypoglycemia in Hyperinsulinemia Hyperammonemia by Inhibiting Glucagon Release
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...
متن کاملMitochondrial GTP insensitivity contributes to hypoglycemia in Hyperinsulinemia Hyperammonemia (HI/HA) by inhibiting glucagon release
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 be...
متن کاملIntraislet hyperinsulinemia prevents the glucagon response to hypoglycemia despite an intact autonomic response.
Because absence of the glucagon response to falling plasma glucose concentrations plays a key role in the pathogenesis of iatrogenic hypoglycemia in patients with insulin-deficient diabetes and the mechanism of this defect is unknown, and given evidence in experimental animals that a decrease in intraislet insulin is a signal to increased glucagon secretion, we examined the role of endogenous i...
متن کامل21 hypoglycemia cases with hyperinsulinemia
Congenital hyperinsulinemic hypoglycemia (CHI) is the common cause of severe hypoglycemia in infancy. Profound hypoglycemia requires appropriate diagnosis and aggressive treatment to prevent severe and irreversible brain damage. Here we report 21 Japanese hypoglycemia cases with severe hyperinsulinemia. We report 16 CHI cases, 2 HIHA cases, 1 GSD1b case and 2 PSS cases. 12 cases of CHI had seve...
متن کاملGlucagon response to hypoglycemia in sympathectomized man.
Hypoglycemia stimulates immunoreactive glucagon (IRG) secretion and increases the activity of the sympathetic nervous system. To ascertain if the augmented alpha cell activity evoked by glucopenia is mediated by the adrenergic nervous system, the glucagon response to insulin-induced hypoglycemia of five subjects with neurologically complete cervical transections resulting from trauma, thereby d...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Diabetes
سال: 2014
ISSN: 0012-1797,1939-327X
DOI: 10.2337/db14-0783