The acute and chronic effects of 20 mM glucose and 10 microM carbachol on beta-cell responses were investigated. Acute exposure of rat islets to 20 mM glucose increased glucose usage rates and resulted in a large insulin-secretory response during a dynamic perifusion. The secretory, but not the metabolic, effect of 20 mM glucose was abolished by simultaneous exposure to 100 microM diazoxide. Gl...

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...

BACKGROUND Up to now, only limited data on long-term medical treatment in congenital hyperinsulinism (CHI) is available. Moreover, most of the drugs used in CHI are therefore not approved. We aimed to assemble more objective information on medical treatment in CHI with regard to type and duration, dosage as well as side effects. METHODS We searched MEDLINE (from 1947) and EMBASE (from 1988) u...

UNLABELLED The aim of this paper was to investigate the effects of pinacidil and diazoxide, the activators of sarcolemmal and mitochondrial ATP-sensitive K(+) channels (K(ATP)), respectively, on heart rate and force of contraction in female and male guinea pigs. Experiments were performed on the isolated, spontaneously beating, right atria obtained from guinea pigs of both genders. First series...

OBJECTIVE Congenital hyperinsulinism (CHI) is a rare but severe cause of hypoglycemia. The present study investigates the clinical presentation, therapeutic outcomes and genetic mutations of CHI in Chinese individuals over the past 15 years. METHODS The authors retrospectively reviewed one case in their department and 206 cases reported from January 2002 to October 2016 in China. PubMed, Ovid...

Iron importer divalent metal transporter 1 (DMT1) plays a crucial role in the nigal iron accumulation in Parkinson's disease (PD). Membrane hyperpolarization is one of the factors that could affect its iron transport function. Besides iron, selective activation of the ATP-sensitive potassium (KATP) channels also contributes to the vulnerability of dopaminergic neurons in PD. Interestingly, acti...

We examined the effect of cardioprotective agents on three distinct phases of the H2O2-induced response that leads to loss of mitochondrial membrane potential (DeltaPsi(m)) and cell death in cultured cardiac myocytes: (1) priming, consisting of calcium-dependent morphological changes in mitochondria (swelling and loss of cristae), with preserved DeltaPsi(m), (2) depolarization, the rapid DeltaP...

We are studying a Turkish family with autosomal-dominant hypertension and brachydactyly; affected persons die of stroke before 50 years of age. With interphase fluorescence in situ hybridization, we found a chromosome 12p deletion, reinsertion, and inversion in affected persons. This finding suggested that the hypertension could be caused by one or more of 3 genes, the ATP-dependent potassium c...

Context The rarest genetic form of congenital hyperinsulinism (HI) has been associated with dominant inactivating mutations in uncoupling protein 2 (UCP2), a mitochondrial inner membrane carrier that modulates oxidation of glucose vs amino acids. Objective To evaluate the frequency of UCP2 mutations in children with HI and phenotypic features of this form of HI. Design We examined 211 child...