The episodic ataxias.

The primary episodic ataxias (EAs) are a group of autosomal-dominant disorders characterized by transient recurrent incoordination and truncal instability, often triggered by physical exertion or emotional stress and variably associated with progressive baseline ataxia. There are now nine designated subtypes EA1-9 (OMIM) and late onset cerebellar ataxia with episodic features as newly designated SCA27B, based largely on genetic loci. Mutations have been identified in multiple individuals and families in 4 of the 9 EA subtypes, mostly with the onset before adulthood.

A secondary mechanism of action for triazole antifungals in Aspergillus fumigatus mediated by hmg1.

Triazole antifungals function as ergosterol biosynthesis inhibitors and are frontline therapy for invasive fungal infections, such as invasive aspergillosis. The primary mechanism of action of triazoles is through the specific inhibition of a cytochrome P450 14-α-sterol demethylase enzyme, Cyp51A/B, resulting in depletion of cellular ergosterol. Here, we uncover a clinically relevant secondary mechanism of action for triazoles within the ergosterol biosynthesis pathway.

An Evaluation of Barriers and Facilitators for a Pressure Injury Prevention Link Nurse Role: A Mixed-Methods Study in New Zealand.

Purpose: The purpose of this study was to identify barriers and facilitators for nurses in a pressure injury prevention (PIP) link nurse role.

Design: Mixed-methods study that used nominal group technique with focus groups, followed by online surveys.

Sample And Setting: The PIP link nurse project recruited 52 RNs; 32 completed the PIP educational program and participated in data collection.

US workers' willingness to accept meatpacking jobs amid the COVID-19 pandemic.

We implement a discrete choice experiment to examine the impact of COVID-19 exposure risk, unemployment risk, enhanced and extended unemployment benefits, and job attributes on low-skilled workers' willingness to accept (WTA) meatpacking jobs. With a sample average WTA wage of $22.77/h, the current national average meatpacking wage of approximately $15/h is too low for these workers to consider this employment opportunity.

Do Past Experience and Group Heterogeneity Matter to Consumer Preferences? Evidence From a Choice Experiment in Urban China.

This study uses a discrete choice experiment to examine consumers' preferences for Fuji apple product attributes and willingness to pay (WTP) estimates for consumers in six cities in China. We estimated the preference heterogeneity by linking the stated preference choice data with consumers' past experience and socioeconomic characteristics in the latent class model. The empirical results show that, first, the past experience variables are crucial in explaining consumer preferences and WTP.

Mechanism of stress-induced attacks in an episodic neurologic disorder.

Stress is the most common trigger among episodic neurologic disorders. In episodic ataxia type 2 (EA2), physical or emotional stress causes episodes of severe motor dysfunction that manifest as ataxia and dystonia. We used the (tg/tg) mouse, a faithful animal model of EA2, to dissect the mechanisms underlying stress-induced motor attacks.

A survey of nurses prescribing in diabetes care: Practices, barriers and facilitators in New Zealand and the United Kingdom.

Aims And Objectives: To compare diabetes-related prescribing practices, barriers and facilitators amongst nurse prescribers in New Zealand and the United Kingdom.

Background: Nurses have been prescribing in the United Kingdom for many years but nurse prescribing in New Zealand is relatively recent. It is unknown whether similar system factors act to facilitate or limit prescribing.

Climate change alters temporal dynamics of alpine soil microbial functioning and biogeochemical cycling via earlier snowmelt.

Soil microbial communities regulate global biogeochemical cycles and respond rapidly to changing environmental conditions. However, understanding how soil microbial communities respond to climate change, and how this influences biogeochemical cycles, remains a major challenge. This is especially pertinent in alpine regions where climate change is taking place at double the rate of the global average, with large reductions in snow cover and earlier spring snowmelt expected as a consequence.

Labor Issues in the Food Supply Chain Amid the COVID-19 Pandemic.

The impacts of COVID-19 on labor in the food supply chain and on workers' decisions to accept essential jobs are discussed. We then analyze surveys administered to low-skilled domestic workers before and during the pandemic to assess respondents' attitudes toward food production, guest workers, immigration policy, and the government's response to COVID-19. Results suggest the outbreak resulted in respondents, on average, shifting their view toward food being a national security issue and a higher degree of empathy for H-2A workers.

High throughput method for measuring urease activity in soil.

Extracellular enzymes break down soil organic matter into smaller compounds and their measurement has proved to be a powerful tool to evaluate the functionality of soils. Urease is the enzyme that degrades urea and is widely considered to be a good proxy of nitrogen (N) mineralisation. But the methods available to measure this enzyme are time consuming; as such, urease is not commonly included in standard enzyme profiling of soils.

Identification of a unique Ca-binding site in rat acid-sensing ion channel 3.

Acid-sensing ion channels (ASICs) evolved to sense changes in extracellular acidity with the divalent cation calcium (Ca) as an allosteric modulator and channel blocker. The channel-blocking activity is most apparent in ASIC3, as removing Ca results in channel opening, with the site's location remaining unresolved. Here we show that a ring of rat ASIC3 (rASIC3) glutamates (Glu435), located above the channel gate, modulates proton sensitivity and contributes to the formation of the elusive Ca block site.

Examining the sustainability potential of a multisite pilot to integrate alcohol screening and brief intervention within three primary care systems.

The U.S. Preventive Services Task Force recommends that clinicians adopt universal alcohol screening and brief intervention as a routine preventive service for adults, and efforts are underway to support its widespread dissemination.

Qualitative comparison of polyethylene terephthalate flakes from various collection systems in Germany.

In 2003, a deposit system for one-way packaging was introduced in Germany. Since that time, polyethylene terephthalate beverage packaging is collected through various collection systems, a deposit system, a reusable packaging system and the 'Green Dot' (the dual system) with the yellow bag. The manner of collection had a decisive influence on the quality of the generated recycled materials.

5-(N, N-Hexamethylene) amiloride is a GABA-A ρ1 receptor positive allosteric modulator.

Guanidine compounds act as ion channel modulators. In the case of Cys-loop receptors, the guanidine compound amiloride antagonized the heteromeric GABA-A, glycine, and nicotinic acetylcholine receptors. However, amiloride exhibits characteristics consistent with a positive allosteric modulator for the human GABA-A (hGABA-A) ρ1 receptor.

Cryptic diversity in Black rats of the Galápagos Islands, Ecuador.

Human activity has facilitated the introduction of a number of alien mammal species to the Galápagos Archipelago. Understanding the phylogeographic history and population genetics of invasive species on the Archipelago is an important step in predicting future spread and designing effective management strategies. In this study, we describe the invasion pathway of across the Galápagos using microsatellite data, coupled with historical knowledge.

Amiloride and GMQ Allosteric Modulation of the GABA-A ρ1 Receptor: Influences of the Intersubunit Site.

Amiloride, a diuretic used in the treatment of hypertension and congestive heart failure, and 2-guanidine-4-methylquinazoline (GMQ) are guanidine compounds that modulate acid-sensing ion channels. Both compounds have demonstrated affinity for a variety of membrane proteins, including members of the Cys-loop family of ligand-gated ion channels, such as the heteromeric GABA-A αβγ receptors. The actions of these guanidine compounds on the homomeric GABA-A ρ1 receptor remains unclear, especially in light of how many GABA-A αβγ receptor modulators have different effects in the GABA-A ρ1 receptors.

CNS myelination requires cytoplasmic dynein function.

Background: Cytoplasmic dynein provides the main motor force for minus-end-directed transport of cargo on microtubules. Within the vertebrate central nervous system (CNS), proliferation, neuronal migration, and retrograde axon transport are among the cellular functions known to require dynein. Accordingly, mutations of DYNC1H1, which encodes the heavy chain subunit of cytoplasmic dynein, have been linked to developmental brain malformations and axonal pathologies.

Minimising methodological biases to improve the accuracy of partitioning soil respiration using natural abundance 13C.

Rationale: Microbial degradation of soil organic matter (heterotrophic respiration) is a key determinant of net ecosystem exchange of carbon, but it is difficult to measure because the CO2 efflux from the soil surface is derived not only from heterotrophic respiration, but also from plant root and rhizosphere respiration (autotrophic). Partitioning total CO2 efflux can be achieved using the different natural abundance stable isotope ratios (δ(13)C) of root and soil CO2. Successful partitioning requires very accurate measurements of total soil efflux δ(13)CO2 and the δ(13)CO2 of the autotrophic and heterotrophic sources, which typically differ by just 2-8‰.

Screening and brief intervention for risky alcohol use.

Alcohol screening and brief intervention are effective evidence-based methods recommended by multiple major medical societies. Primary care providers are uniquely positioned to recognize patients with alcohol use problems when they utilize screening in their practices. SBI has the potential to improve patients' overall health, reduce the risk of alcohol dependence, and prevent future alcohol-exposed pregnancies.