Normal vision and development in mice with low functional expression of Kir7.1 in heterozygosis for a blindness-producing mutation inactivating the channel.

K channel Kir7.1 expressed at the apical membrane of the retinal pigment epithelium (RPE) plays an essential role in retinal function. An isoleucine-to-threonine mutation at position 120 of the protein is responsible for blindness-causing vitreo-retinal dystrophy.

Differentiation of the motor cost associated with cognitive tasks in Parkinson's disease: A dual-task study.

Parkinson's disease is a neurodegenerative condition associated with motor and cognitive impairments. While the execution of dual cognitive-motor tasks imposes a cost on gait velocity, it has been barely determined if the gait deterioration depends on the specific cognitive domain involved in the dual-task. Twenty-four subjects (12 patients with Parkinson's disease and 12 healthy subjects) carried out a single task (gait alone) and several dual tasks where the concurrent second task was the Trail Making Test (Part A) and the six tasks of the Frontal Assessment Battery.

Altered phosphatidylinositol regulation of mutant inwardly rectifying K Kir7.1 channels associated with inherited retinal degeneration disease.

Key Points: Kir7.1 K channel expressed in retinal pigment epithelium is mutated in inherited retinal degeneration diseases. We study Kir7.

Kir7.1 inwardly rectifying K channel is expressed in ciliary body non pigment epithelial cells and might contribute to intraocular pressure regulation.

Inwardly rectifying K channel Kir7.1 is expressed in epithelia where it shares membrane localisation with the Na/K-pump. The ciliary body epithelium (CBE) of the eye is a determinant of intraocular pressure (IOP) through NaCl-driven fluid secretion of aqueous humour.

The Effects of Respiratory Muscle Training on Phonatory Measures in Individuals with Parkinson's Disease.

Introduction: In individuals with Parkinson's disease (PD), respiratory muscle weakness and rigidity, bradykinesia of abdominal muscles and stiffness of the chest wall, affect the respiratory component of voice intensity due to reduced pulmonary capacity and airflow needed to vibrate the vocal folds. It may be possible to improve voice production by strengthening respiratory muscles. The purpose of this study was to evaluate the effects of inspiratory and expiratory muscle training on voice production outcomes in individuals with PD.

A novel Kir7.1 splice variant expressed in various mouse tissues shares organisational and functional properties with human Leber amaurosis-causing mutations of this K channel.

Kir7.1 is an inwardly rectifying K channel present in epithelia where it shares membrane localization with the Na/K-pump. In the present communication we report the presence of a novel splice variant of Kir7.

The effects of respiratory muscle training on peak cough flow in patients with Parkinson's disease: a randomized controlled study.

Objective: To compare the effects of an inspiratory versus and expiratory muscle-training program on voluntary and reflex peak cough flow in patients with Parkinson disease.

Design: A randomized controlled study.

Setting: Home-based training program.

Tissue Distribution of Kir7.1 Inwardly Rectifying K Channel Probed in a Knock-in Mouse Expressing a Haemagglutinin-Tagged Protein.

Kir7.1 encoded by the gene in the mouse is an inwardly rectifying K channel present in epithelia where it shares membrane localization with the Na/K-pump. Further investigations of the localisation and function of Kir7.

Colony Expansion of Socially Motile Myxococcus xanthus Cells Is Driven by Growth, Motility, and Exopolysaccharide Production.

Myxococcus xanthus, a model organism for studies of multicellular behavior in bacteria, moves exclusively on solid surfaces using two distinct but coordinated motility mechanisms. One of these, social (S) motility is powered by the extension and retraction of type IV pili and requires the presence of exopolysaccharides (EPS) produced by neighboring cells. As a result, S motility requires close cell-to-cell proximity and isolated cells do not translocate.

Identification and functional expression of a glutamate- and avermectin-gated chloride channel from Caligus rogercresseyi, a southern Hemisphere sea louse affecting farmed fish.

Parasitic sea lice represent a major sanitary threat to marine salmonid aquaculture, an industry accounting for 7% of world fish production. Caligus rogercresseyi is the principal sea louse species infesting farmed salmon and trout in the southern hemisphere. Most effective control of Caligus has been obtained with macrocyclic lactones (MLs) ivermectin and emamectin.

Rapid recycling of ClC-2 chloride channels between plasma membrane and endosomes: role of a tyrosine endocytosis motif in surface retrieval.

ClC-2 chloride channel is present in the brain and some transporting epithelia where its function is poorly understood. We have now demonstrated that the surface channels are rapidly internalised and approximately the 70% of the surface membrane protein recycles after 4- to 8-min internalisation. Endocytosis of ClC-2 was dependent upon tyrosine 179 located within an endocytic motif.

A genetically encoded ratiometric sensor to measure extracellular pH in microdomains bounded by basolateral membranes of epithelial cells.

Extracellular pH, especially in relatively inaccessible microdomains between cells, affects transport membrane protein activity and might have an intercellular signaling role. We have developed a genetically encoded extracellular pH sensor capable of detecting pH changes in basolateral spaces of epithelial cells. It consists of a chimerical membrane protein displaying concatenated enhanced variants of cyan fluorescence protein (ECFP) and yellow fluorescence protein (EYFP) at the external aspect of the cell surface.

Basolateral localization of native ClC-2 chloride channels in absorptive intestinal epithelial cells and basolateral sorting encoded by a CBS-2 domain di-leucine motif.

The Cl- channel ClC-2 is expressed in transporting epithelia and has been proposed as an alternative route for Cl- efflux that might compensate for the malfunction of CFTR in cystic fibrosis. There is controversy concerning the cellular and membrane location of ClC-2, particularly in intestinal tissue. The aim of this paper is to resolve this controversy by immunolocalization studies using tissues from ClC-2 knockout animals as control, ascertaining the sorting of ClC-2 in model epithelial cells and exploring the possible molecular signals involved in ClC-2 targeting.

Functional evaluation of human ClC-2 chloride channel mutations associated with idiopathic generalized epilepsies.

The ClC-2 Cl- channel has been postulated to play a role in the inhibitory GABA response in neurons or to participate in astrocyte-dependent extracellular electrolyte homeostasis. Three different mutations in the CLCN2 gene, encoding the voltage-dependent homodimeric ClC-2 channel, have been associated with idiopathic generalized epilepsy (IGE). We study their function in vitro by patch clamp and confocal microscopy in transiently transfected HEK-293 cells.

ClC-2 in guinea pig colon: mRNA, immunolabeling, and functional evidence for surface epithelium localization.

The principal function of the colon in fluid homeostasis is the absorption of NaCl and water. Apical membrane Na(+) channels, Na(+)/H(+) and Cl(-)/HCO exchangers, have all been postulated to mediate NaCl entry into colonocytes. The identity of the basolateral exit pathway for Cl(-) is unknown.