Exercise Training and Neurodegeneration in Mitochondrial Disorders: Insights From the Harlequin Mouse.

Aim: Cerebellar neurodegeneration is a main phenotypic manifestation of mitochondrial disorders caused by apoptosis-inducing factor (AIF) deficiency. We assessed the effects of an exercise training intervention at the cerebellum and brain level in a mouse model (Harlequin, ) of AIF deficiency.

Methods: Male wild-type (WT) and mice were assigned to an exercise (Ex) or control (sedentary [Sed]) group ( = 10-12/group).

Minimum Electromyographic Burst Duration in Healthy Controls: Implications for Electrodiagnosis in Movement Disorders.

Background: Electromyogram (EMG) burst duration can provide additional diagnostic information when investigating hyperkinetic movement disorders, particularly when a functional movement disorder is suspected. It is generally accepted that EMG bursts <50 milliseconds are pathological.

Objective: To reassess minimum physiological EMG burst duration.

Single cell fluorescence imaging of glycan uptake by intestinal bacteria.

Microbes in the intestines of mammals degrade dietary glycans for energy and growth. The pathways required for polysaccharide utilization are functionally diverse; moreover, they are unequally dispersed between bacterial genomes. Hence, assigning metabolic phenotypes to genotypes remains a challenge in microbiome research.

High molecular weight hyaluronic acid: a two-pronged protectant against infection of the urogenital tract?

Objectives: Recurrent urinary tract infections are associated with uropathogenic (UPEC) ascending and infecting the urinary tract. Antibiotics provide only symptomatic relief, not prevent recurrence. Clinical evidence suggests that intravesical glycosaminoglycan therapy, such as hyaluronic acid (HA), helps reduce UTI recurrence.

A Large Polysaccharide Produced by Helicobacter hepaticus Induces an Anti-inflammatory Gene Signature in Macrophages.

Interactions between the host and its microbiota are of mutual benefit and promote health. Complex molecular pathways underlie this dialog, but the identity of microbe-derived molecules that mediate the mutualistic state remains elusive. Helicobacter hepaticus is a member of the mouse intestinal microbiota that is tolerated by the host.

Cough and bronchiectasis.

Bronchiectasis is a chronic lung disease with permanent airway dilatation, mucus retention and recurrent lower respiratory tract infections. Bronchiectasis is increasing in prevalence and has a significant morbidity and an excess mortality rate over age matched controls. It is increasingly identified during investigations into chronic cough and is evident high resolution CT scanning.

Assessment of epilepsy using noninvasive visual psychophysics tests of surround suppression.

Powerful endogenous inhibitory mechanisms are thought to restrict the spread of epileptic discharges in cortical networks. Similar inhibitory mechanisms also influence physiological processing. We reasoned, therefore, that useful information about the quality of inhibitory restraint in individuals with epilepsy may be gleaned from psychophysical assays of these physiological processes.

The Association Between Sleep Duration, Insulin Sensitivity, and β-Cell Function: The EGIR-RISC Study.

Context: In the past decade, over 3 dozen studies reported a relationship between self-reported short sleep and disturbed glucose metabolism. A study with insulin sensitivity assessed according to the gold standard hyperinsulinemic-euglycemic clamp is, however, still missing.

Objective: To evaluate the cross-sectional association of sleep duration with insulin sensitivity and β-cell function in the European group for the study of insulin resistance (EGIR-RISC) study cohort.

The Contribution of Non-catalytic Carbohydrate Binding Modules to the Activity of Lytic Polysaccharide Monooxygenases.

Lignocellulosic biomass is a sustainable industrial substrate. Copper-dependent lytic polysaccharide monooxygenases (LPMOs) contribute to the degradation of lignocellulose and increase the efficiency of biofuel production. LPMOs can contain non-catalytic carbohydrate binding modules (CBMs), but their role in the activity of these enzymes is poorly understood.

Oxidative stress--a key emerging impact factor in health, ageing, lifestyle and aesthetics.

Oxidative stress is the resultant damage that arises due to redox imbalances, more specifically an increase in destructive free radicals and reduction in protection from antioxidants and the antioxidant defence pathways. Oxidation of lipids by reactive oxygen species (ROS) can damage cellular structures and result in premature cell death. At low levels, ROS-induced oxidative stress can be prevented through the action of antioxidants, however, when ROS are present in excess, inflammation and cytotoxicity eventually results leading to cellular oxidative stress damage.

The GH130 Family of Mannoside Phosphorylases Contains Glycoside Hydrolases That Target β-1,2-Mannosidic Linkages in Candida Mannan.

The depolymerization of complex glycans is an important biological process that is of considerable interest to environmentally relevant industries. β-Mannose is a major component of plant structural polysaccharides and eukaryotic N-glycans. These linkages are primarily cleaved by glycoside hydrolases, although recently, a family of glycoside phosphorylases, GH130, have also been shown to target β-1,2- and β-1,4-mannosidic linkages.

Salvaging hope: Is increasing NAD(+) a key to treating mitochondrial myopathy?

Mitochondrial diseases can arise from mutations either in mitochondrial DNA or in nuclear DNA encoding mitochondrially destined proteins. Currently, there is no cure for these diseases although treatments to ameliorate a subset of the symptoms are being developed. In this issue of EMBO Molecular Medicine, Khan et al (2014) use a mouse model to test the efficacy of a simple dietary supplement of nicotinamide riboside to treat and prevent mitochondrial myopathies.

Surgical endoscopy training: the Joint Advisory Group on gastrointestinal endoscopy national review.

Background And Aims: Endoscopy performance is dependent on the technical ability and experience of the operator. There is anxiety among surgical trainees that certification to perform independent endoscopy to agreed national standards by the date of award of certificate of completion of training is not achievable. The aim of this study was to evaluate the delivery of endoscopy training to UK-based general surgery trainees.