Superconductivity in AuNiGe Ohmic contacts to a GaAs-based high mobility two-dimensional electron gas

To cool a high mobility two-dimensional electron gas (2DEG) at a GaAs-AlGaAs heterojunction to milliKelvin temperatures, we have fabricated low resistance Ohmic contacts based on alloys of Au, Ni, and Ge. The Ohmic contacts have a typical contact resistance of R C ≈ 0.8 ω at 4.2 K, which drops to 0.2 ω below 0.9 K. Scanning electron microscope images establish that the contacts have the same inhomogeneous microstructure that has been observed in previous studies. Measurements of the contact resistance R C, the four-terminal resistance along the top of a single contact, and the vertical resistance RV all show that there is a superconductor in the Ohmic contact, which can be turned completely normal with a magnetic field of 0.15 T. We briefly discuss how this superconductivity may be affecting the electrical transport measurements of 2DEGs, especially how it may hinder the cooling of electrons in a 2DEG below 0.1 K

Max Müller and the Comparative Method

This is an Author’s Accepted Manuscript of an article published by Edinburgh University Press in Comparative Critical Studies. The Version of Record is available online at: https://www.euppublishing.com/doi/10.3366/ccs.2015.016

Clinical impact of genomic testing in patients with suspected monogenic kidney disease

Purpose: To determine the diagnostic yield and clinical impact of exome sequencing (ES) in patients with suspected monogenic kidney disease. Methods: We performed clinically accredited singleton ES in a prospectively ascertained cohort of 204 patients assessed in multidisciplinary renal genetics clinics at four tertiary hospitals in Melbourne, Australia. Results: ES identified a molecular diagnosis in 80 (39%) patients, encompassing 35 distinct genetic disorders. Younger age at presentation was independently associated with an ES diagnosis (p < 0.001). Of those diagnosed, 31/80 (39%) had a change in their clinical diagnosis. ES diagnosis was considered to have contributed to management in 47/80 (59%), including negating the need for diagnostic renal biopsy in 10/80 (13%), changing surveillance in 35/80 (44%), and changing the treatment plan in 16/80 (20%). In cases with no change to management in the proband, the ES result had implications for the management of family members in 26/33 (79%). Cascade testing was subsequently offered to 40/80 families (50%). Conclusion: In this pragmatic pediatric and adult cohort with suspected monogenic kidney disease, ES had high diagnostic and clinical utility. Our findings, including predictors of positive diagnosis, can be used to guide clinical practice and health service design

Bioenergetic status modulates motor neuron vulnerability and pathogenesis in a zebrafish model of spinal muscular atrophy

Degeneration and loss of lower motor neurons is the major pathological hallmark of spinal muscular atrophy (SMA), resulting from low levels of ubiquitously-expressed survival motor neuron (SMN) protein. One remarkable, yet unresolved, feature of SMA is that not all motor neurons are equally affected, with some populations displaying a robust resistance to the disease. Here, we demonstrate that selective vulnerability of distinct motor neuron pools arises from fundamental modifications to their basal molecular profiles. Comparative gene expression profiling of motor neurons innervating the extensor digitorum longus (disease-resistant), gastrocnemius (intermediate vulnerability), and tibialis anterior (vulnerable) muscles in mice revealed that disease susceptibility correlates strongly with a modified bioenergetic profile. Targeting of identified bioenergetic pathways by enhancing mitochondrial biogenesis rescued motor axon defects in SMA zebrafish. Moreover, targeting of a single bioenergetic protein, phosphoglycerate kinase 1 (Pgk1), was found to modulate motor neuron vulnerability in vivo. Knockdown of pgk1 alone was sufficient to partially mimic the SMA phenotype in wild-type zebrafish. Conversely, Pgk1 overexpression, or treatment with terazosin (an FDA-approved small molecule that binds and activates Pgk1), rescued motor axon phenotypes in SMA zebrafish. We conclude that global bioenergetics pathways can be therapeutically manipulated to ameliorate SMA motor neuron phenotypes in vivo

The political rationalities of fair trade consumption in the United Kingdom

This article situates the analysis of fair-trade consumption in the context ofdebates about civic activism and political participation. It argues that fair-tradeconsumption should be understood as a political phenomenon, which, through themediating action of organizations and campaigns, makes claims on states, corporations,and institutions. This argument is made by way of a case study ofTraidcraft, a key player in the fair-trade movement in the United Kingdom. Thestudy focuses on how Traidcraft approaches and enrolls its supporters