(Q37634376)

21 August 2017

1 reference

title

K(ATP) channelopathies in the pancreas (English)

1 reference

21 August 2017

0 references

Maria S Remedi

1

1 reference

21 August 2017

Joseph C Koster

series ordinal

2

1 reference

21 August 2017

publication date

18 November 2009

1 reference

21 August 2017

1 reference

21 August 2017

volume

460

1 reference

21 August 2017

issue

2

1 reference

21 August 2017

page(s)

307-320

1 reference

https://scigraph.springernature.com/pub.10.1007/s00424-009-0756-x

21 August 2017

exact match

0 references

cites work

Glibenclamide unresponsiveness in a Brazilian child with permanent neonatal diabetes mellitus and DEND syndrome due to a C166Y mutation in KCNJ11 (Kir6.2) gene

1 reference

Crossref

https://api.crossref.org/works/10.1007%2FS00424-009-0756-X

Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism

21 January 2018

1 reference

12 December 2020

Activating mutations in the gene encoding the ATP-sensitive potassium-channel subunit Kir6.2 and permanent neonatal diabetes

1 reference

12 December 2020

KCNJ11 activating mutations are associated with developmental delay, epilepsy and neonatal diabetes syndrome and other neurological features.

1 reference

12 December 2020

Functional analyses of novel mutations in the sulfonylurea receptor 1 associated with persistent hyperinsulinemic hypoglycemia of infancy

1 reference

12 December 2020

Current status of the E23K Kir6.2 polymorphism: implications for type-2 diabetes

1 reference

12 December 2020

Genome-wide association analysis identifies loci for type 2 diabetes and triglyceride levels

1 reference

12 December 2020

The G53D mutation in Kir6.2 (KCNJ11) is associated with neonatal diabetes and motor dysfunction in adulthood that is improved with sulfonylurea therapy

1 reference

12 December 2020

Activation of the ATP-sensitive K+ channel by long chain acyl-CoA. A role in modulation of pancreatic beta-cell glucose sensitivity.

1 reference

12 December 2020

Improved motor development and good long-term glycaemic control with sulfonylurea treatment in a patient with the syndrome of intermediate developmental delay, early-onset generalised epilepsy and neonatal diabetes associated with the V59M mutation i

1 reference

12 December 2020

The essential role of the Walker A motifs of SUR1 in K-ATP channel activation by Mg-ADP and diazoxide

1 reference

12 December 2020

Association and stoichiometry of K(ATP) channel subunits

1 reference

12 December 2020

Mutations in the ABCC8 gene encoding the SUR1 subunit of the KATP channel cause transient neonatal diabetes, permanent neonatal diabetes or permanent diabetes diagnosed outside the neonatal period

1 reference

12 December 2020

Suppression of KATP channel activity protects murine pancreatic beta cells against oxidative stress

1 reference

12 December 2020

SUR, ABC proteins targeted by KATP channel openers.

1 reference

12 December 2020

Insulin secretagogues, sulfonylurea receptors and K(ATP) channels

1 reference

12 December 2020

Neonatal diabetes mellitus: a disease linked to multiple mechanisms

1 reference

12 December 2020

Sulphonylurea treatment does not improve psychomotor development in children with KCNJ11 mutations causing permanent neonatal diabetes mellitus accompanied by developmental delay and epilepsy (DEND syndrome).

1 reference

12 December 2020

Molecular mechanism for ATP-dependent closure of the K+ channel Kir6.2

1 reference

12 December 2020

Sequence variations in the human Kir6.2 gene, a subunit of the beta-cell ATP-sensitive K-channel: no association with NIDDM in while Caucasian subjects or evidence of abnormal function when expressed in vitro

1 reference

12 December 2020

Activating mutations in the ABCC8 gene in neonatal diabetes mellitus

1 reference

12 December 2020

Replication of genome-wide association signals in UK samples reveals risk loci for type 2 diabetes

1 reference

12 December 2020

Sulfonylurea treatment outweighs insulin therapy in short-term metabolic control of patients with permanent neonatal diabetes mellitus due to activating mutations of the KCNJ11 (KIR6.2) gene

1 reference

12 December 2020

Mechanisms by which glucose can control insulin release independently from its action on adenosine triphosphate-sensitive K+ channels in mouse B cells

1 reference

12 December 2020

ATP-sensitive K+ channels in pancreatic beta-cells. Spare-channel hypothesis.

1 reference

12 December 2020

The molecular basis of the specificity of action of K(ATP) channel openers

1 reference

12 December 2020

Targeted Overactivity of β Cell KATP Channels Induces Profound Neonatal Diabetes

1 reference

12 December 2020

MgATP activates the beta cell KATP channel by interaction with its SUR1 subunit.

1 reference

12 December 2020

Signaling in channel/enzyme multimers: ATPase transitions in SUR module gate ATP-sensitive K+ conductance

1 reference

12 December 2020

Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates

1 reference

12 December 2020

Spontaneous recovery from hyperglycemia by regeneration of pancreatic beta-cells in Kir6.2G132S transgenic mice.

1 reference

12 December 2020

Reconstitution of IKATP: an inward rectifier subunit plus the sulfonylurea receptor

1 reference

12 December 2020

Kir6.2 mutations causing neonatal diabetes provide new insights into Kir6.2-SUR1 interactions

1 reference

12 December 2020

Opposite effects of tolbutamide and diazoxide on the ATP-dependent K+ channel in mouse pancreatic beta-cells

1 reference

12 December 2020

A novel method for measurement of submembrane ATP concentration.

1 reference

12 December 2020

Adenosine diphosphate as an intracellular regulator of insulin secretion.

1 reference

12 December 2020

Sulfonylurea improves CNS function in a case of intermediate DEND syndrome caused by a mutation in KCNJ11.

1 reference

12 December 2020

Coexpression of the type 2 diabetes susceptibility gene variants KCNJ11 E23K and ABCC8 S1369A alter the ATP and sulfonylurea sensitivities of the ATP-sensitive K(+) channel

1 reference

12 December 2020

Mechanism of improvement in glucose metabolism after chronic glyburide therapy

1 reference

12 December 2020

The E23K variant of Kir6.2 associates with impaired post-OGTT serum insulin response and increased risk of type 2 diabetes

1 reference

12 December 2020

Switching from insulin to oral sulfonylureas in patients with diabetes due to Kir6.2 mutations.

1 reference

12 December 2020

Relapsing diabetes can result from moderately activating mutations in KCNJ11.

1 reference

12 December 2020

K(IR)6.2 polymorphism predisposes to type 2 diabetes by inducing overactivity of pancreatic beta-cell ATP-sensitive K(+) channels

1 reference

12 December 2020

Mutations at the same residue (R50) of Kir6.2 (KCNJ11) that cause neonatal diabetes produce different functional effects.

1 reference

12 December 2020

Mechanism of action of a sulphonylurea receptor SUR1 mutation (F132L) that causes DEND syndrome.

1 reference

12 December 2020

Dysregulation of insulin secretion in children with congenital hyperinsulinism due to sulfonylurea receptor mutations

1 reference

12 December 2020

Oscillations of membrane potential and cytosolic Ca(2+) concentration in SUR1(-/-) beta cells

1 reference

12 December 2020

A review of islet of Langerhans degeneration in rodent models of type 2 diabetes

1 reference

12 December 2020

Candidate gene association study in type 2 diabetes indicates a role for genes involved in beta-cell function as well as insulin action

1 reference

12 December 2020

Concentration dependence and time course of the effects of glucose on adenine and guanine nucleotides in mouse pancreatic islets

1 reference

12 December 2020

Secondary consequences of beta cell inexcitability: identification and prevention in a murine model of K(ATP)-induced neonatal diabetes mellitus

1 reference

12 December 2020

Tolerance for ATP-insensitive K(ATP) channels in transgenic mice

1 reference

12 December 2020

A new subtype of autosomal dominant diabetes attributable to a mutation in the gene for sulfonylurea receptor 1.

1 reference

12 December 2020

Large-scale association studies of variants in genes encoding the pancreatic beta-cell KATP channel subunits Kir6.2 (KCNJ11) and SUR1 (ABCC8) confirm that the KCNJ11 E23K variant is associated with type 2 diabetes

1 reference

12 December 2020

Toward linking structure with function in ATP-sensitive K+ channels

1 reference

12 December 2020

Sulfonylureas correct trafficking defects of disease-causing ATP-sensitive potassium channels by binding to the channel complex

1 reference

12 December 2020

Triggering and amplifying pathways of regulation of insulin secretion by glucose

1 reference

12 December 2020

Intracellular ATP directly blocks K+ channels in pancreatic B-cells

1 reference

12 December 2020

Review. SUR1: a unique ATP-binding cassette protein that functions as an ion channel regulator.

1 reference

12 December 2020

UKPDS 26: Sulphonylurea failure in non-insulin-dependent diabetic patients over six years. UK Prospective Diabetes Study (UKPDS) Group.

1 reference

12 December 2020

Monogenic diabetes in children and young adults: Challenges for researcher, clinician and patient

1 reference

12 December 2020

Kir6.2 variant E23K increases ATP-sensitive K+ channel activity and is associated with impaired insulin release and enhanced insulin sensitivity in adults with normal glucose tolerance

1 reference

12 December 2020

ATP4- mediates closure of pancreatic beta-cell ATP-sensitive potassium channels by interaction with 1 of 4 identical sites

1 reference

12 December 2020

Kir6.2 polymorphisms sensitize beta-cell ATP-sensitive potassium channels to activation by acyl CoAs: a possible cellular mechanism for increased susceptibility to type 2 diabetes?

1 reference

12 December 2020

Increased ATPase activity produced by mutations at arginine-1380 in nucleotide-binding domain 2 of ABCC8 causes neonatal diabetes

1 reference

12 December 2020

Neonatal diabetes mellitus

1 reference

12 December 2020

Octameric stoichiometry of the KATP channel complex

1 reference

12 December 2020

A mutation (R826W) in nucleotide-binding domain 1 of ABCC8 reduces ATPase activity and causes transient neonatal diabetes

1 reference

12 December 2020

KATP channels as molecular sensors of cellular metabolism

1 reference

12 December 2020

Mutations in ATP-Sensitive K+ Channel Genes Cause Transient Neonatal Diabetes and Permanent Diabetes in Childhood or Adulthood

1 reference

12 December 2020

An adenylate kinase is involved in KATP channel regulation of mouse pancreatic beta cells

1 reference

12 December 2020

Genetics of congenital hyperinsulinism

1 reference

12 December 2020

Permanent neonatal diabetes due to mutations in KCNJ11 encoding Kir6.2: patient characteristics and initial response to sulfonylurea therapy

1 reference

12 December 2020

ATPase activity of the sulfonylurea receptor: a catalytic function for the KATP channel complex.

1 reference

12 December 2020

KATP channel inhibition by ATP requires distinct functional domains of the cytoplasmic C terminus of the pore-forming subunit

1 reference

12 December 2020

Sur domains that associate with and gate KATP pores define a novel gatekeeper

1 reference

12 December 2020

Beta-cell proliferation and apoptosis in the developing normal human pancreas and in hyperinsulinism of infancy

1 reference

12 December 2020

Structural determinants of PIP(2) regulation of inward rectifier K(ATP) channels

1 reference

12 December 2020

Functional effects of KCNJ11 mutations causing neonatal diabetes: enhanced activation by MgATP.

1 reference

12 December 2020

Defective trafficking and function of KATP channels caused by a sulfonylurea receptor 1 mutation associated with persistent hyperinsulinemic hypoglycemia of infancy

1 reference

12 December 2020

Functional analysis of a structural model of the ATP-binding site of the KATP channel Kir6.2 subunit.

1 reference

12 December 2020

Successful sulfonylurea treatment of an insulin-naïve neonate with diabetes mellitus due to a KCNJ11 mutation

1 reference

12 December 2020

Functional analysis of six Kir6.2 (KCNJ11) mutations causing neonatal diabetes.

1 reference

12 December 2020

Cytosolic adenylate kinases regulate K-ATP channel activity in human beta-cells

1 reference

12 December 2020

Identification of residues contributing to the ATP binding site of Kir6.2.

1 reference

12 December 2020

Differential nucleotide regulation of KATP channels by SUR1 and SUR2A.

1 reference

12 December 2020

Direct Photoaffinity Labeling of the Kir6.2 Subunit of the ATP-sensitive K+Channel by 8-Azido-ATP

1 reference

12 December 2020

Glucagon-Like Peptide 1(7-36) Amide Stimulates Exocytosis in Human Pancreatic -Cells by Both Proximal and Distal Regulatory Steps in Stimulus-Secretion Coupling

1 reference

12 December 2020

Dominantly inherited hyperinsulinism caused by a mutation in the sulfonylurea receptor type 1.

1 reference

12 December 2020

An ATP-binding mutation (G334D) in KCNJ11 is associated with a sulfonylurea-insensitive form of developmental delay, epilepsy, and neonatal diabetes.

1 reference

12 December 2020

Glucagon-like peptide-1 modulates Ca2+ current but not K+ATP current in intact mouse pancreatic B-cells

1 reference

12 December 2020

The C42R mutation in the Kir6.2 (KCNJ11) gene as a cause of transient neonatal diabetes, childhood diabetes, or later-onset, apparently type 2 diabetes mellitus.

1 reference

12 December 2020

Molecular determinants of KATP channel inhibition by ATP.

1 reference

12 December 2020

Cloning of the beta cell high-affinity sulfonylurea receptor: a regulator of insulin secretion

1 reference

12 December 2020

Abnormalities of pancreatic islets by targeted expression of a dominant-negative KATP channel

1 reference

12 December 2020

Genetic variations in the pancreatic ATP-sensitive potassium channel, beta-cell dysfunction, and susceptibility to type 2 diabetes

1 reference

12 December 2020

Cell-type specific expression of ATP-sensitive potassium channels in the rat hippocampus.

1 reference

12 December 2020

Glucose generates sub-plasma membrane ATP microdomains in single islet beta-cells. Potential role for strategically located mitochondria.

1 reference

12 December 2020

Glucose action 'beyond ionic events' in the pancreatic beta cell

1 reference

12 December 2020

The gating of nucleotide-sensitive K+ channels in insulin-secreting cells can be modulated by changes in the ratio ATP4-/ADP3- and by nonhydrolyzable derivatives of both ATP and ADP.

1 reference

12 December 2020

Expression of ATP-insensitive KATP channels in pancreatic beta-cells underlies a spectrum of diabetic phenotypes

1 reference

12 December 2020

Mechanism of cloned ATP-sensitive potassium channel activation by oleoyl-CoA

1 reference

12 December 2020

PIP2 and PIP as determinants for ATP inhibition of KATP channels

1 reference

12 December 2020

Sur1 knockout mice. A model for K(ATP) channel-independent regulation of insulin secretion

1 reference

12 December 2020

Update of mutations in the genes encoding the pancreatic beta-cell K(ATP) channel subunits Kir6.2 (KCNJ11) and sulfonylurea receptor 1 (ABCC8) in diabetes mellitus and hyperinsulinism

1 reference

12 December 2020

Sulfonylurea receptor type 1 knock-out mice have intact feeding-stimulated insulin secretion despite marked impairment in their response to glucose

1 reference

12 December 2020

3-D structural and functional characterization of the purified KATP channel complex Kir6.2-SUR1.

1 reference

12 December 2020

Molecular mechanisms of neonatal hyperinsulinism

1 reference

12 December 2020

A Kir6.2 mutation causing severe functional effects in vitro produces neonatal diabetes without the expected neurological complications

1 reference

12 December 2020

Missense mutations in the pancreatic islet beta cell inwardly rectifying K+ channel gene (KIR6.2/BIR): a meta-analysis suggests a role in the polygenic basis of Type II diabetes mellitus in Caucasians

1 reference

12 December 2020

Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy

1 reference

12 December 2020

A novel mutation causing DEND syndrome: a treatable channelopathy of pancreas and brain.

1 reference

12 December 2020

Functional effects of naturally occurring KCNJ11 mutations causing neonatal diabetes on cloned cardiac KATP channels

1 reference

12 December 2020

Hyperinsulinism induced by targeted suppression of beta cell KATP channels

1 reference

12 December 2020

Tandem function of nucleotide binding domains confers competence to sulfonylurea receptor in gating ATP-sensitive K+ channels.

1 reference

12 December 2020

Diabetes and insulin secretion: the ATP-sensitive K+ channel (K ATP) connection

1 reference

12 December 2020

Chronic antidiabetic sulfonylureas in vivo: reversible effects on mouse pancreatic beta-cells

1 reference

12 December 2020

N-terminal transmembrane domain of the SUR controls trafficking and gating of Kir6 channel subunits

1 reference

12 December 2020

Electrophysiology of the pancreatic beta-cell

1 reference

12 December 2020

Mutations in the sulonylurea receptor gene are associated with familial hyperinsulinism in Ashkenazi Jews.

1 reference

12 December 2020

ATP and sulfonylurea sensitivity of mutant ATP-sensitive K+ channels in neonatal diabetes: implications for pharmacogenomic therapy

1 reference

12 December 2020

Hyperinsulinism in infancy: from basic science to clinical disease.

1 reference

12 December 2020

A Mutation in the TMD0-L0 Region of Sulfonylurea Receptor-1 (L225P) Causes Permanent Neonatal Diabetes Mellitus (PNDM)

1 reference

12 December 2020

Identification and pharmacological correction of a membrane trafficking defect associated with a mutation in the sulfonylurea receptor causing familial hyperinsulinism

1 reference

12 December 2020

A minimum of fuel is necessary for tolbutamide to mimic the effects of glucose on electrical activity in pancreatic beta-cells.

1 reference

12 December 2020

Expression of an activating mutation in the gene encoding the KATP channel subunit Kir6.2 in mouse pancreatic beta cells recapitulates neonatal diabetes

1 reference

12 December 2020

Defective insulin secretion and enhanced insulin action in KATP channel-deficient mice.

1 reference

12 December 2020

The kinetic and physical basis of K(ATP) channel gating: toward a unified molecular understanding

1 reference

12 December 2020

Molecular basis of Kir6.2 mutations associated with neonatal diabetes or neonatal diabetes plus neurological features

1 reference

12 December 2020

Macrosomia and hyperinsulinaemic hypoglycaemia in patients with heterozygous mutations in the HNF4A gene

1 reference

12 December 2020

Cell-type specific depression of neuronal excitability in rat hippocampus by activation of ATP-sensitive potassium channels.

1 reference

12 December 2020

Glucose induces closure of single potassium channels in isolated rat pancreatic beta-cells

1 reference

12 December 2020

Oscillations of secretion driven by oscillations of cytoplasmic Ca2+ as evidences in single pancreatic islets

1 reference

12 December 2020

Nucleotide sensitivity of pancreatic ATP-sensitive potassium channels and type 2 diabetes

1 reference

12 December 2020

ATP-sensitive potassium channelopathies: focus on insulin secretion

1 reference

12 December 2020

A rare mutation in ABCC8/SUR1 leading to altered ATP-sensitive K+ channel activity and beta-cell glucose sensing is associated with type 2 diabetes in adults

1 reference

12 December 2020

Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel mutations

1 reference

12 December 2020

Genetics and pathophysiology of hyperinsulinism in infancy.

1 reference

12 December 2020

Truncation of Kir6.2 produces ATP-sensitive K+ channels in the absence of the sulphonylurea receptor

1 reference

12 December 2020

Haplotype structure and genotype-phenotype correlations of the sulfonylurea receptor and the islet ATP-sensitive potassium channel gene region

1 reference

12 December 2020

10.1007/S00424-009-0756-X

Identifiers

DOI

1 reference

21 August 2017

1 reference