نتایج جستجو برای: kcnj11

تعداد نتایج: 484  

Journal: :Philosophical Transactions of the Royal Society B: Biological Sciences 2008

Background & Objective: The incidence of diabetes in liver transplant recipients is one of the common problems among these patients witha negative effect on the success of the transplant and the patient's life. Post Transplant Diabetes Mellitus (PTDM) has many similarities with type 2 diabetes from pathophysiplogical, clinical, and genetic viewpoints... One of the genotypes involved in PTD...

2015
Raphael Del Roio Liberatore Priscila Manzini Ramos Gil Guerra Thais Della Manna Ivani Novato Silva Carlos Eduardo Martinelli

OBJECTIVE To study the clinical and molecular characteristics of a sample of Brazilian patients with Congenital Hyperinsulinemic Hypoglycemia (CHH). METHODS Electronic message was sent to members from Endocrinology Department- Brazilian Society of Pediatrics requesting clinical data for all cases of CHH. A whole blood sample from living patients was requested for DNA extraction followed by a ...

Journal: :Human molecular genetics 2006
Peter Proks Amanda L Arnold Jan Bruining Christophe Girard Sarah E Flanagan Brian Larkin Kevin Colclough Andrew T Hattersley Frances M Ashcroft Sian Ellard

Neonatal diabetes is a genetically heterogeneous disorder with nine different genetic aetiologies reported to date. Heterozygous activating mutations in the KCNJ11 gene encoding Kir6.2, the pore-forming subunit of the ATP-sensitive potassium (K(ATP)) channel, are the most common cause of permanent neonatal diabetes. The sulphonylurea receptor (SUR) SUR1 serves as the regulatory subunit of the K...

2017
Ja Hyang Cho Eungu Kang Beom Hee Lee Gu Hwan Kim Jin Ho Choi Han Wook Yoo

Permanent neonatal diabetes mellitus (PNDM) is caused by mutations in the ATP-sensitive potassium channel (KATP channel) subunits. Developmental delay, epilepsy, and neonatal diabetes (DEND) syndrome is the most severe form of PNDM and is characterized by various neurologic features. We report on a patient with DEND syndrome following initial misdiagnosis with type 1 DM, who was successfully sw...

Journal: :Diabetes 2006
Kenju Shimomura Christophe A J Girard Peter Proks Joanna Nazim Jonathan D Lippiat Franco Cerutti Renata Lorini Sian Ellard Andrew T Hattersley Fabrizio Barbetti Frances M Ashcroft

Heterozygous mutations in the human Kir6.2 gene (KCNJ11), the pore-forming subunit of the ATP-sensitive K(+) channel (K(ATP) channel), are a common cause of neonatal diabetes. We identified a novel KCNJ11 mutation, R50Q, that causes permanent neonatal diabetes (PNDM) without neurological problems. We investigated the functional effects this mutation and another at the same residue (R50P) that l...

2017
E. De Franco R. Caswell J. A. L. Houghton V. Iotova A. T. Hattersley S. Ellard

AIMS An early genetic diagnosis of neonatal diabetes guides clinical management and results in improved treatment in ~ 40% of patients. In the offspring of individuals with neonatal diabetes, a prenatal diagnosis allows accurate estimation of the risk of developing diabetes and, eventually, the most appropriate treatment for the baby. In this study, we performed non-invasive prenatal genetic te...

2013
Matthijs L. Becker Ewan R. Pearson Ivan Tkáč

Oral antidiabetic drugs (OADs) are used for more than a half-century in the treatment of type 2 diabetes. Only in the last five years, intensive research has been conducted in the pharmacogenetics of these drugs based mainly on the retrospective register studies, but only a handful of associations detected in these studies were replicated. The gene variants in CYP2C9, ABCC8/KCNJ11, and TCF7L2 w...

2013
Maria Carla Proverbio Eleonora Mangano Alessandra Gessi Roberta Bordoni Roberta Spinelli Rosanna Asselta Paola Sogno Valin Stefania Di Candia Ilaria Zamproni Cecilia Diceglie Stefano Mora Manuela Caruso-Nicoletti Alessandro Salvatoni Gianluca De Bellis Cristina Battaglia

Congenital hyperinsulinism of infancy (CHI) is a rare disorder characterized by severe hypoglycemia due to inappropriate insulin secretion. The genetic causes of CHI have been found in genes regulating insulin secretion from pancreatic β-cells; recessive inactivating mutations in the ABCC8 and KCNJ11 genes represent the most common events. Despite the advances in understanding the molecular pat...

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