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Open AccessCorrection: Differential effects of mutations of POPDC proteins on heteromeric interaction and membrane trafficking
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Article
Open AccessDifferential effects of mutations of POPDC proteins on heteromeric interaction and membrane trafficking
The Popeye domain containing (POPDC) genes encode sarcolemma-localized cAMP effector proteins. Mutations in blood vessel epicardial substance (BVES) also known as POPDC1 and POPDC2 have been associated with limb-...
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Article
Open AccessEnhanced firing of locus coeruleus neurons and SK channel dysfunction are conserved in distinct models of prodromal Parkinson’s disease
Parkinson’s disease (PD) is clinically defined by the presence of the cardinal motor symptoms, which are associated with a loss of dopaminergic nigrostriatal neurons in the substantia nigra pars compacta (SNpc...
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Article
Open AccessDisease-associated HCN4 V759I variant is not sufficient to impair cardiac pacemaking
The hyperpolarization-activated cation current If is a key determinant for cardiac pacemaker activity. It is conducted by subunits of the hyperpolarization-activated cyclic nucleotide–gated (HCN) channel family, ...
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Article
A lower X-gate in TASK channels traps inhibitors within the vestibule
TWIK-related acid-sensitive potassium (TASK) channels—members of the two pore domain potassium (K2P) channel family—are found in neurons1, cardiomyocytes2–4 and vascular smooth muscle cells5, where they are invol...
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Article
Electrophysiological characterization of a large set of novel variants in the SCN5A-gene: identification of novel LQTS3 and BrS mutations
SCN5A encodes for the α-subunit of the cardiac voltage-gated sodium channel Nav1.5. Gain-of-function mutations in SCN5A are related to congenital long QT syndrome (LQTS3) characterized by...
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Article
Open AccessFunctional mutagenesis screens reveal the ‘cap structure’ formation in disulfide-bridge free TASK channels
Two-pore-domain potassium (K2P) channels have a large extracellular cap structure formed by two M1-P1 linkers, containing a cysteine for dimerization. However, this cysteine is not present in the TASK-1/3/5 subfa...
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Article
The role of acid-sensitive two-pore domain potassium channels in cardiac electrophysiology: focus on arrhythmias
The current kinetics of two-pore domain potassium (K2P) channels resemble those of the steady-state K+ currents being active during the plateau phase of cardiac action potentials. Recent studies support that K2P ...
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Article
Kv1.5 blockers preferentially inhibit TASK-1 channels: TASK-1 as a target against atrial fibrillation and obstructive sleep apnea?
Atrial fibrillation and obstructive sleep apnea are responsible for significant morbidity and mortality in the industrialized world. There is a high medical need for novel drugs against both diseases, and here...
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Article
A splice variant of the two-pore domain potassium channel TREK-1 with only one pore domain reduces the surface expression of full-length TREK-1 channels
We have identified a novel splice variant of the human and rat two-pore domain potassium (K2P) channel TREK-1. The splice variant TREK-1e results from skip** of exon 5, which causes a frame shift in exon 6. The...
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Article
Soluble polysialylated NCAM: a novel player of the innate immune system in the lung
Posttranslational modification of the neural cell adhesion molecule (NCAM) by polysialic acid (polySia) is well studied in the nervous system and described as a dynamic modulator of plastic processes like prec...
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Article
Side pockets provide the basis for a new mechanism of Kv channel–specific inhibition
A Kv1 channel inhibitor and potential therapeutic lead achieves selectivity by binding both the conserved central cavity and newly identified side pockets, which provide the key determinants for channel specif...
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Article
Non dominant-negative KCNJ2 gene mutations leading to Andersen-Tawil syndrome with an isolated cardiac phenotype
Andersen-Tawil syndrome (ATS) is characterized by dysmorphic features, periodic paralyses and abnormal ventricular repolarization. After genoty** a large set of patients with congenital long-QT syndrome, we ...
