A Research Project to Investigate the Impact of Electricity System Requirements On the Design and Optimal Application of the Powerformer (TM)

This paper describes the aims and the methodology of a major collaborative research project between the University of Queensland and Australian and Swedish industry partners, including ABB Corporate Research (Sweden), Alstom Power (Sweden and Australia), PowerLink Queensland, Stanwell Corporation, C S Energy and Tarong Energy. The project is investigating the likely benefits of significance to the Queensland system, which will arise from the optimisation of the new Powerformer technology for the generation of electricity at transmission or sub-transmission voltages, i.e. without step-up transformers

Loss-of-Function Variants in HOPS Complex Genes VPS16 and VPS41 Cause Early Onset Dystonia Associated with Lysosomal Abnormalities.

OBJECTIVES: The majority of people with suspected genetic dystonia remain undiagnosed after maximal investigation, implying that a number of causative genes have not yet been recognized. We aimed to investigate this paucity of diagnoses. METHODS: We undertook weighted burden analysis of whole-exome sequencing (WES) data from 138 individuals with unresolved generalized dystonia of suspected genetic etiology, followed by additional case-finding from international databases, first for the gene implicated by the burden analysis (VPS16), and then for other functionally related genes. Electron microscopy was performed on patient-derived cells. RESULTS: Analysis revealed a significant burden for VPS16 (Fisher's exact test p value, 6.9 × 109 ). VPS16 encodes a subunit of the homotypic fusion and vacuole protein sorting (HOPS) complex, which plays a key role in autophagosome-lysosome fusion. A total of 18 individuals harboring heterozygous loss-of-function VPS16 variants, and one with a microdeletion, were identified. These individuals experienced early onset progressive dystonia with predominant cervical, bulbar, orofacial, and upper limb involvement. Some patients had a more complex phenotype with additional neuropsychiatric and/or developmental comorbidities. We also identified biallelic loss-of-function variants in VPS41, another HOPS-complex encoding gene, in an individual with infantile-onset generalized dystonia. Electron microscopy of patient-derived lymphocytes and fibroblasts from both patients with VPS16 and VPS41 showed vacuolar abnormalities suggestive of impaired lysosomal function. INTERPRETATION: Our study strongly supports a role for HOPS complex dysfunction in the pathogenesis of dystonia, although variants in different subunits display different phenotypic and inheritance characteristics. ANN NEUROL 2020;88:867-877

The CGR4 lightning sensor

The design of the CGR4 lightning sensor is based on several observed characteristics of the electric field changes caused by nearby lightning, and the radiofrequency noise bursts from lightning. The electric field signals from lightning are detected by a simple aerial, and processed by a combination of analogue and digital circuitry under microprocessor control to determine if there is a nearby lightning flash, and, if so, to assign the type of flash as negative ground flash (NGF), positive ground flash (PGF), or cloud flash (CF). These types have distinctive features in their electric field change waveforms, enabling an assignment of type of lightning to be made by obtaining several measures of the waveform characteristics and applying an appropriate set of rules. A method of estimating the sensor's effective range and area has been developed using data for a set of nearby lightning events. This involves the recording of the variation of electric field with time, the response of a lightning sensor, and the distance to the lightning flash using thunder ranging. As the sensor is able to apply different threshold overall field changes for each type of lightning detected, a single effective range of 11.3 km (effective area 400 km) for all types of lightning was achieved by using threshold field changes of 520 volts per metre (V/m) for CF, 760 V/m for NGF, and 1200 V/m for PGF. The sensor responds to about 90 per cent of all flashes at 8 km, about 40 per cent at 11 km, and approaching zero above 16 km

Search for new diagnostics for metal oxide surge arrester

A new diagnostic technique called “return voltage measurement” is investigated to assess its effectiveness for monitoring degradation in metal oxide blocks. The return voltage measurement was conducted on a number of distribution class zinc-oxide arresters. The findings from these measurements are described in this paper. The first part describes some results on the reproducibility of return voltage measurements and also the polarity effects on measurements. The second part presents the results of monitoring the arresters while being subjected to elevated temperatures using the return voltage measurement