Lottie Lyell

Lottie Lyell was a much-loved silent film star in Australia. She was also a scenario writer, director, editor, producer, and art director. Quietly working alongside director Raymond Longford, she had a significant influence on the twenty-eight films they made together. Lyell starred in nearly all the films, but it is now generally accepted that she contributed a great deal more than was officially acknowledged at the time (Wright 1986, 1-14; Pike and Cooper 1998, 19, 109; Dooley 2000, 4)

Biallelic Variants in PYROXD2 Cause a Severe Infantile Metabolic Disorder Affecting Mitochondrial Function

Pyridine Nucleotide-Disulfide Oxidoreductase Domain 2 (PYROXD2; previously called YueF) is a mitochondrial inner membrane/matrix-residing protein and is reported to regulate mitochondrial function. The clinical importance of PYROXD2 has been unclear, and little is known of the protein’s precise biological function. In the present paper, we report biallelic variants in PYROXD2 identified by genome sequencing in a patient with suspected mitochondrial disease. The child presented with acute neurological deterioration, unresponsive episodes, and extreme metabolic acidosis, and received rapid genomic testing. He died shortly after. Magnetic resonance imaging (MRI) brain imaging showed changes resembling Leigh syndrome, one of the more common childhood mitochondrial neurological diseases. Functional studies in patient fibroblasts showed a heightened sensitivity to mitochondrial metabolic stress and increased mitochondrial superoxide levels. Quantitative proteomic analysis demonstrated decreased levels of subunits of the mitochondrial respiratory chain complex I, and both the small and large subunits of the mitochondrial ribosome, suggesting a mitoribosomal defect. Our findings support the critical role of PYROXD2 in human cells, and suggest that the biallelic PYROXD2 variants are associated with mitochondrial dysfunction, and can plausibly explain the child’s clinical presentation

Some Issues in the Application of Competition Policy to Agriculture

National Competition Policy aims to further micro-economic reform by creating a more competitive environment for both industry and government activity. In 1995 Australian Governments agreed to the National Competition Policy package, which included the Competition Principles Agreement. To meet its commitments, the NSW Government has actively applied the principles of the agreement to its various portfolios, including agriculture. In this paper, comments are provided in process issues associated with implementation of the Competition Principles Agreement that have influenced reform outcomes. Comments are then provided on several specific issues arising from the application of the agreement to agriculture, including the identification and measurement of public benefits and costs