OBJECTIVE: Because children with congenital hyperinsulinism (HI) caused by recessive loss of function mutations in the adenosine triphosphate (ATP)-dependent potassium channel (K(ATP)-HI) are not leucine sensitive, we evaluated for protein-induced hypoglycemia with oral protein tolerance tests. STUDY DESIGN: Blood glucose and insulin concentrations were measured every 15 minutes for 3 hours after an oral protein load in children with K(ATP)-HI (n = 11) and compared with those of children with glutamate dehydrogenase HI (n = 12) and control subjects (n = 12). RESULTS: Similar to children with glutamate dehydrogenase HI, patients with K(ATP)-HI displayed protein-induced hypoglycemia (10/11) with blood glucose concentrations declining by 17 to 69 mg/dL. In contrast, oral protein had little effect on blood glucose concentrations in control subjects. CONCLUSIONS: Protein-induced hypoglycemia is a feature of K(ATP)-HI, despite the absence of leucine sensitivity. The results indicate that amino acids can stimulate insulin secretion via a glutamate dehydrogenase- and K(ATP) channel-independent pathway.
OBJECTIVE: Because children with congenital hyperinsulinism (HI) caused by recessive loss of function mutations in the adenosine triphosphate (ATP)-dependent potassium channel (K(ATP)-HI) are not leucine sensitive, we evaluated for protein-induced hypoglycemia with oral protein tolerance tests. STUDY DESIGN:Blood glucose and insulin concentrations were measured every 15 minutes for 3 hours after an oral protein load in children with K(ATP)-HI (n = 11) and compared with those of children with glutamate dehydrogenase HI (n = 12) and control subjects (n = 12). RESULTS: Similar to children with glutamate dehydrogenase HI, patients with K(ATP)-HI displayed protein-induced hypoglycemia (10/11) with blood glucose concentrations declining by 17 to 69 mg/dL. In contrast, oral protein had little effect on blood glucose concentrations in control subjects. CONCLUSIONS: Protein-induced hypoglycemia is a feature of K(ATP)-HI, despite the absence of leucine sensitivity. The results indicate that amino acids can stimulate insulin secretion via a glutamate dehydrogenase- and K(ATP) channel-independent pathway.
Authors: Changhong Li; Amanda M Ackermann; Kara E Boodhansingh; Tricia R Bhatti; Chengyang Liu; Jonathan Schug; Nicolai Doliba; Bing Han; Karen E Cosgrove; Indraneel Banerjee; Franz M Matschinsky; Itzhak Nissim; Klaus H Kaestner; Ali Naji; N Scott Adzick; Mark J Dunne; Charles A Stanley; Diva D De León Journal: Diabetes Date: 2017-04-25 Impact factor: 9.461
Authors: Sara E Pinney; Karthik Ganapathy; Jonathan Bradfield; David Stokes; Ariella Sasson; Katarzyna Mackiewicz; Kara Boodhansingh; Nkecha Hughes; Susan Becker; Stephanie Givler; Courtney Macmullen; Dimitrios Monos; Arupa Ganguly; Hakon Hakonarson; Charles A Stanley Journal: Horm Res Paediatr Date: 2013-07-13 Impact factor: 2.852
Authors: Changhong Li; Itzhak Nissim; Pan Chen; Carol Buettger; Habiba Najafi; Yevgeny Daikhin; Ilana Nissim; Heather W Collins; Marc Yudkoff; Charles A Stanley; Franz M Matschinsky Journal: J Biol Chem Date: 2008-04-29 Impact factor: 5.157
Authors: Diva D De León; Changhong Li; Madeleine I Delson; Franz M Matschinsky; Charles A Stanley; Doris A Stoffers Journal: J Biol Chem Date: 2008-07-17 Impact factor: 5.157