Early life metal dysregulation in amyotrophic lateral sclerosis.

Objective: Deficiencies and excess of essential elements and toxic metals are implicated in amyotrophic lateral sclerosis (ALS), but the age when metal dysregulation appears remains unknown. This study aims to determine whether metal uptake is dysregulated during childhood in individuals eventually diagnosed with ALS.

Methods: Laser ablation-inductively coupled plasma-mass spectrometry was used to obtain time series data of metal uptake using biomarkers in teeth from autopsies or dental extractions of ALS (n = 36) and control (n = 31) participants.

A randomized, double-blind, placebo-controlled trial of testosterone for treatment of postmenopausal women with aromatase inhibitor-induced arthralgias: Alliance study A221102.

Purpose: To evaluate the efficacy of testosterone supplementation for improving aromatase inhibitor musculoskeletal symptoms (AIMSS).

Methods: Postmenopausal women experiencing moderate-to-severe arthralgias while taking adjuvant aromatase inhibitors for breast cancer were enrolled in this trial. Initially, patients were randomly allocated to receive either a subcutaneous testosterone pellet versus a placebo pellet.

Lagged WQS regression for mixtures with many components.

The developmental timing of exposures to toxic chemicals or combinations of chemicals may be as important as the dosage itself. This concept is called "critical windows of exposure." The time boundaries of such windows can be detected if exposure data are collected repeatedly in short time intervals.

Modulation of Small Artery Function by Insulin in Young Women: Role of Adiposity.

Objectives: Vascular dysfunction is common in obesity. Insulin can directly modulate arterial function, but its role is unclear in obesity. We examined the influence of adiposity on direct effects of insulin on human artery responses.

Dysregulated biodynamics in metabolic attractor systems precede the emergence of amyotrophic lateral sclerosis.

Evolutionarily conserved mechanisms maintain homeostasis of essential elements, and are believed to be highly time-variant. However, current approaches measure elemental biomarkers at a few discrete time-points, ignoring complex higher-order dynamical features. To study dynamical properties of elemental homeostasis, we apply laser ablation inductively-coupled plasma mass spectrometry (LA-ICP-MS) to tooth samples to generate 500 temporally sequential measurements of elemental concentrations from birth to 10 years.

Dendritic cell-derived hepcidin sequesters iron from the microbiota to promote mucosal healing.

Bleeding and altered iron distribution occur in multiple gastrointestinal diseases, but the importance and regulation of these changes remain unclear. We found that hepcidin, the master regulator of systemic iron homeostasis, is required for tissue repair in the mouse intestine after experimental damage. This effect was independent of hepatocyte-derived hepcidin or systemic iron levels.

Biological clocks and incremental growth line formation in dentine.

Dentine- and enamel-forming cells secrete matrix in consistent rhythmic phases, resulting in the formation of successive microscopic growth lines inside tooth crowns and roots. Experimental studies of various mammals have proven that these lines are laid down in subdaily, daily (circadian), and multidaily rhythms, but it is less clear how these rhythms are initiated and maintained. In 2001, researchers reported that lesioning the so-called master biological clock, the suprachiasmatic nucleus (SCN), halted daily line formation in rat dentine, whereas subdaily lines persisted.

The Fundamental Characteristics of a Translational Scientist.

Translational science is defined as the field of investigation focused on understanding the scientific and operational principles underlying each step of the translational process. Further development of the field is advanced by describing the key desirable characteristics of individuals who seek to uncover these principles to increase the efficiency and efficacy of translation. The members of Translation Together, a newly launched international collaborative effort to advance translational innovation, present here a consensus representation of the fundamental characteristics of a translational scientist.

A new species of Cyrtodactylus Gray (Gekkonidae: Squamata) from Manus Island, and extended description and range extension for Cyrtodactylus sermowaiensis (De Rooij).

Systematic investigations of vertebrate faunas from the islands of Melanesia are revealing high levels of endemism, dynamic biogeographic histories, and in some cases surprisingly long evolutionary histories of insularity. The bent-toed geckos in the Cyrtodactylus sermowaiensis Group mainly occur in northern New Guinea and nearby islands, however a further isolated population occurs on Manus Island in the Admiralty Archipelago approximately 300 km to the north of New Guinea. Here we first present a review of the genetic diversity, morphological variation and distribution of Cyrtodactylus sermowaiensis from northern New Guinea.

Dataset for sequencing and assembly of the European endangered white-clawed crayfish () abdominal muscle transcriptome.

The white-clawed crayfish () is an endangered species in Europe with limited genomic information. Despite its conservation status there is no transcriptomic data available for in public databases. The data here represents the first transcriptome profile of the white-clawed crayfish generated using Illumina stranded RNA sequencing.

Improved genomic resources for the black tiger prawn (Penaeus monodon).

World production of farmed crustaceans was 7.8 million tons in 2016. While only making up approximately 10% of world aquaculture production, crustaceans are generally high-value species and can earn significant export income for producing countries.

Flight responses of eastern gray kangaroos to benign or harmful human behavior.

Globally, wilderness is being converted for rural and agricultural land use. In countryside landscapes, many habitat structures remain intact, providing suitable habitat for wildlife species that can accurately assess novel risks and develop tolerance to benign disturbances. Associative learning that promotes avoidance and also facilitates desensitization to benign disturbance is key to persisting in these landscapes.

Publisher Correction: Therapies for rare diseases: therapeutic modalities, progress and challenges ahead.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Hybrid trans-apical atrioventricular valve-in-valve implantation in Fontan circulation.

Trans-apical approach has been proved successful in failing surgical bio-prosthesis in both mitral and aortic position in adult patients. Recently, valve-in-valve treatments have been applied even in patients with complex congenital heart disease. Here, we report the case of a 32 years old lady with left atrial isomerism, complete AV septal defect, interrupted inferior vena cava with azygos continuation who underwent Kawashima procedure with atrial Fontan.

Quantum-Enhanced Advanced LIGO Detectors in the Era of Gravitational-Wave Astronomy.

The Laser Interferometer Gravitational Wave Observatory (LIGO) has been directly detecting gravitational waves from compact binary mergers since 2015. We report on the first use of squeezed vacuum states in the direct measurement of gravitational waves with the Advanced LIGO H1 and L1 detectors. This achievement is the culmination of decades of research to implement squeezed states in gravitational-wave detectors.

Therapies for rare diseases: therapeutic modalities, progress and challenges ahead.

Most rare diseases still lack approved treatments despite major advances in research providing the tools to understand their molecular basis, as well as legislation providing regulatory and economic incentives to catalyse the development of specific therapies. Addressing this translational gap is a multifaceted challenge, for which a key aspect is the selection of the optimal therapeutic modality for translating advances in rare disease knowledge into potential medicines, known as orphan drugs. With this in mind, we discuss here the technological basis and rare disease applicability of the main therapeutic modalities, including small molecules, monoclonal antibodies, protein replacement therapies, oligonucleotides and gene and cell therapies, as well as drug repurposing.

Changes in child functioning pre-to post-neuropsychological evaluation.

Although a key goal of neuropsychological evaluation is to improve patient outcomes, research regarding changes in child functioning following neuropsychological evaluation is very limited. This project examined changes from before neuropsychological evaluation to several months afterward in parent report of their child's functioning and ability to participate across home, school, and community settings. Fifty-one parents of children and adolescents receiving their first outpatient neuropsychological evaluation completed questionnaires prior to initial parent interview and following the final report (of n = 162 who were mailed the follow-up questionnaire).