- ►diabetesjournals.org A Kelly, C Li, Z Gao, CA Stanley, FM … - Diabetes, 2002 - Am Diabetes Assoc Identification of regulatory mutations of glutamate dehydrogenase (GDH) in a
form of congenital hyperinsulinism (GDH-HI) is providing a model for basal
insulin secretion (IS) and amino acid (AA)-stimulated insulin secretion ... Cited by 21 - Related articles - BL Direct - All 4 versions
JC Fournet, C Junien - Hormone research, 2000 - content.karger.com Congenital hyperinsulinism (CHI) is the most important cause of persistent
hypoglycaemia in the neonate and infant. It is a clinically and genetically
heterogeneous entity. The clinical heterogeneity is manifested by severity ... Cited by 22 - Related articles - BL Direct - All 6 versions
- ►diabetesjournals.org T Otonkoski, N Kaminen, J Ustinov, R Lapatto, … - Diabetes, 2003 - Am Diabetes Assoc We have identified patients in whom strenuous physical exercise leads to
hypoglycemia caused by inappropriate insulin release (exercise-induced
hyperinsulinism [EIHI]). The aim of the present study was to test the ... Cited by 34 - Related articles - BL Direct - All 7 versions
B Dekel, D Lubin, D Modan-Moses, J Quint, B … - Clinical pediatrics, 2002 - cpj.sagepub.com Co ongenital hyperinsulin- ism or persistent hyperin- sulinemic hypoglycemia of
infancy (PHHI) is character- ized by an inappropriate over- ... Departments of
1Pediatrics and 2Neonatology, Sheba Medical Center, The Sackler Faculty of ... Cited by 7 - Related articles - BL Direct - All 3 versions
T Meissner, B Friedmann, JG Okun, MA Schwab … - Hormone and Metabolic Research, 2005 - 万方数据资源系统 Exercise-induced hyperinsulinism (EIHI) is a recently described entity
characterised by recurrent episodes of hypoglycaemia induced by physical
exercise. The index patient for this disorder and a matched control were ... Cited by 10 - Related articles - BL Direct - All 7 versions
S Eaton, I Chatziandreou, S Krywawych, S Pen … - Biochemical Society Transactions, 2003 - bst.portlandpress.com Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with
hyperinsulinism: a novel glucose–fatty acid cycle? ... Key words:
b-oxidation, carnitine palmitoyltransferase I (CPT I), 3-hydroxyacyl-CoA ... Cited by 18 - Related articles - Cached - BL Direct - All 11 versions
K Hussain, PT Clayton, S Krywawych, I … - The Journal of pediatrics, 2005 - Elsevier Short-, medium-, and long-chain 3-hydroxyacyl-CoA dehydrogenase activity was
measured in fibroblasts as described previously. 6 The protein was normalized to
supernatant protein rather than total cellular protein. Proteins were ... Cited by 27 - Related articles - All 5 versions
CA Stanley - Molecular genetics and metabolism, 2004 - Elsevier The second most common form of congenital hyperinsulinism, the
hyperinsulinism/hyperammonemia syndrome (HI/HA), is associated with dominantly
expressed missense mutations of the mitochondrial matrix enzyme, glutamate ... Cited by 30 - Related articles - All 4 versions
- ►bmj.com T Meissner, T Otonkoski, R Feneberg, B … - British Medical Journal, 2001 - adc.bmj.com PATIENT 1 A 16 year old boy of Caucasian origin presented with a two year
history of recurrent syncope. These episodes usually occurred a few minutes
after vigorous exercise. Family history was unremarkable. Blood glucose ... Cited by 15 - Related articles - BL Direct - All 11 versions
- ►endojournals.org A Kelly, D Ng, RJ Ferry Jr, A Grimberg, S … - Journal of Clinical Endocrinology & Metabolism, 2001 - Endocrine Soc 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 ... Cited by 37 - Related articles - BL Direct - All 6 versions
