Protecting Wild Land from Wind Farms in a Post-EU Scotland

Scotland is one of the places in Europe to have experienced significant wind farm development over recent years. Concern about impacts on wild land has resulted in legal challenges based on European Union (EU) law. This article analyses whether wild land can be protected from wind farms and the differences that the United Kingdom (UK) departure from the EU will make. It considers the concept of 'wild land' compared with 'wilderness', analyses the legal basis (if any) for wild land protection, and examines potential impacts from wind farms. It highlights the significance of EU environmental law, particularly nature conservation and environmental assessment law, and analyses recent Scottish jurisprudence that has applied this. The role of the European Commission and Court of Justice of the EU (CJEU) is emphasised as a key part of EU environmental law. The article asks whether relevant global and regional environmental agreements can effectively replace the content of the substantive law and context of the Commission and CJEU. Four environmental agreements and two related compliance procedures are briefly evaluated. The conclusion is that while EU law does not directly provide protection for wild land, it is considerably stronger than the international environmental agreements that may replace it

ATAD3 gene cluster deletions cause cerebellar dysfunction associated with altered mitochondrial DNA and cholesterol metabolism

Although mitochondrial disorders are clinically heterogeneous, they frequently involve the central nervous system and are among the most common neurogenetic disorders. Identifying the causal genes has benefited enormously from advances in high-throughput sequencing technologies; however, once the defect is known, researchers face the challenge of deciphering the underlying disease mechanism. Here we characterize large biallelic deletions in the region encoding the ATAD3C, ATAD3B and ATAD3A genes. Although high homology complicates genomic analysis of the ATAD3 defects, they can be identified by targeted analysis of standard single nucleotide polymorphism array and whole exome sequencing data. We report deletions that generate chimeric ATAD3B/ATAD3A fusion genes in individuals from four unrelated families with fatal congenital pontocerebellar hypoplasia, whereas a case with genomic rearrangements affecting the ATAD3C/ATAD3B genes on one allele and ATAD3B/ATAD3A genes on the other displays later-onset encephalopathy with cerebellar atrophy, ataxia and dystonia. Fibroblasts from affected individuals display mitochondrial DNA abnormalities, associated with multiple indicators of altered cholesterol metabolism. Moreover, drug-induced perturbations of cholesterol homeostasis cause mitochondrial DNA disorganization in control cells, while mitochondrial DNA aggregation in the genetic cholesterol trafficking disorder Niemann-Pick type C disease further corroborates the interdependence of mitochondrial DNA organization and cholesterol. These data demonstrate the integration of mitochondria in cellular cholesterol homeostasis, in which ATAD3 plays a critical role. The dual problem of perturbed cholesterol metabolism and mitochondrial dysfunction could be widespread in neurological and neurodegenerative diseases

Genotyping-by-sequencing supports a genetic basis for wing reduction in an alpine New Zealand stonefly

Wing polymorphism is a prominent feature of numerous insect groups, but the genomic basis for this diversity remains poorly understood. Wing reduction is a commonly observed trait in many species of stonefies, particularly in cold or alpine environments. The widespread New Zealand stonefy Zelandoperla fenestrata species group (Z. fenestrata, Z. tillyardi, Z. pennulata) contains populations ranging from fully winged (macropterous) to vestigial-winged (micropterous), with the latter phenotype typically associated with high altitudes. The presence of fightless forms on numerous mountain ranges, separated by lowland fully winged populations, suggests wing reduction has occurred multiple times. We use Genotyping by Sequencing (GBS) to test for genetic diferentiation between fully winged (n=62) and vestigial-winged (n=34) individuals, sampled from a sympatric population of distinct wing morphotypes, to test for a genetic basis for wing morphology. While we found no population genetic diferentiation between these two morphotypes across 6,843 SNP loci, we did detect several outlier loci that strongly diferentiated morphotypes across independent tests. These fndings indicate that small regions of the genome are likely to be highly diferentiated between morphotypes, suggesting a genetic basis for wing reduction. Our results provide a clear basis for ongoing genomic analysis to elucidate critical regulatory pathways for wing development in Pterygota