KATP channels as molecular sensors of cellular metabolism

CG Nichols - Nature, 2006 - nature.com
In responding to cytoplasmic nucleotide levels, ATP-sensitive potassium (KATP) channel
activity provides a unique link between cellular energetics and electrical excitability. Over …

Update of variants identified in the pancreatic β‐cell KATP channel genes KCNJ11 and ABCC8 in individuals with congenital hyperinsulinism and diabetes

E De Franco, C Saint‐Martin, K Brusgaard… - Human …, 2020 - Wiley Online Library
The most common genetic cause of neonatal diabetes and hyperinsulinism is pathogenic
variants in ABCC8 and KCNJ11. These genes encode the subunits of the β‐cell ATP …

Genotype and phenotype correlations in 417 children with congenital hyperinsulinism

KE Snider, S Becker, L Boyajian… - The Journal of …, 2013 - academic.oup.com
Context: Hypoglycemia due to congenital hyperinsulinism (HI) is caused by mutations in 9
genes. Objective: Our objective was to correlate genotype with phenotype in 417 children …

ATP-Sensitive potassium channels and their physiological and pathophysiological roles.

A Tinker, Q Aziz, Y Li, M Specterman - Compr Physiol, 2018 - qmro.qmul.ac.uk
ATP sensitive potassium channels (KATP) are so named because they open as cellular ATP
levels fall. This leads to membrane hyperpolarization and thus links cellular metabolism to …

Update of mutations in the genes encoding the pancreatic beta‐cell KATP channel subunits Kir6.2 (KCNJ11) and sulfonylurea receptor 1 (ABCC8) in diabetes …

SE Flanagan, S Clauin, C Bellanné‐Chantelot… - Human …, 2009 - Wiley Online Library
The beta‐cell ATP‐sensitive potassium (KATP) channel is a key component of stimulus‐
secretion coupling in the pancreatic beta‐cell. The channel couples metabolism to …

The genetic basis of congenital hyperinsulinism

C James, RR Kapoor, D Ismail… - Journal of medical genetics, 2009 - jmg.bmj.com
Congenital hyperinsulinism (CHI) is biochemically characterised by the dysregulated
secretion of insulin from pancreatic β-cells. It is a major cause of persistent …

The genetics of ATP‐binding cassette transporters

M Dean - Methods in enzymology, 2005 - Elsevier
The ATP‐binding cassette (ABC) superfamily consists of membrane proteins that transport a
wide variety of substrates across membranes. Mutations in ABC transporters cause or …

Loss-of-Function ABCC8 Mutations in Pulmonary Arterial Hypertension

MS Bohnen, L Ma, N Zhu, H Qi… - Circulation: Genomic …, 2018 - Am Heart Assoc
Background: In pulmonary arterial hypertension (PAH), pathological changes in pulmonary
arterioles progressively raise pulmonary artery pressure and increase pulmonary vascular …

KATP channel therapeutics at the bedside

A Jahangir, A Terzic - Journal of molecular and cellular cardiology, 2005 - Elsevier
The family of potassium channel openers regroups drugs that share the property of
activating adenosine triphosphate-sensitive potassium (KATP) channels, metabolic sensors …

Mutations in the genes encoding the pancreatic beta‐cell KATP channel subunits Kir6.2 (KCNJ11) and SUR1 (ABCC8) in diabetes mellitus and hyperinsulinism

AL Gloyn, J Siddiqui, S Ellard - Human mutation, 2006 - Wiley Online Library
The beta‐cell ATP‐sensitive potassium channel is a key component of stimulus‐secretion
coupling in the pancreatic beta‐cell. The channel couples metabolism to membrane …