Pathogenic DEGS1 variants have been reported in individuals with autosomal recessive hypomyelinating leukodystrophy 18 (HLD18; MIM# 618404). Here we describe three participants with HLD features and a previously unreported homozygous DEGS1 5′ splice site variant, c.825+4_825 + 5delAGinsTT (NM_003676.4). We used next-generation DNA and transcriptome sequencing, cell-based splicing assays, and tandem mass spectrometry to detect...

The Asia region harbors exceptional chondrichthyan (shark, ray, and chimaera) diversity but faces intense fishing pressure. The Important Shark and Ray Areas (ISRA) process provides a collaborative, evidence-based framework to identify critical habitats and inform spatial management. We assessed ISRAs across the Bay of Bengal, Southeast Asia, and the Northwest Pacific to characterize their extent, ecological significance, and...

Aim Humans have altered ecosystem productivity and biodiversity worldwide by changing land‐cover types and management. High local species richness is commonly found in geographic areas and ecosystems with high net primary productivity (NPP), but the long‐term effects of modification on productivity and biodiversity change, and particularly on the relationship between the two, are poorly understood. Here we evaluate whether hum...

Protected area (PA) networks have in the past been constructed to include all major habitats, but have often been developed through consideration of only a few indicator taxa or across restricted areas, and rarely account for global climate change. Systematic conservation planning (SCP) aims to improve the efficiency of biodiversity conservation, particularly when addressing internationally agreed protection targets. We apply...

The threat posed to protected areas by the illegal killing of wildlife is countered principally by ranger patrols that aim to detect and deter potential offenders. Deterring poaching is a fundamental conservation objective, but its achievement is difficult to identify, especially when the prime source of information comes in the form of the patrols’ own records, which inevitably contain biases. The most common metric of deterr...

Climate change vulnerability assessments are commonly used to identify species at risk from global climate change, but the wide range of methodologies available makes it difficult for end users, such as conservation practitioners or policymakers, to decide which method to use as a basis for decision‐making. In this study, we evaluate whether different assessments consistently assign species to the same risk categories and whet...

Ecologists trying to understand the value of habitat to animals must first describe the value of resources contained in the habitat to animals and, second, they must describe spatial variation in resource quality at a resolution relevant to individual animal foraging. We addressed these issues in a study of koalas ( Phascolarctos cinereus ) in a Eucalyptus woodland. We measured beneficial and deterrent chemical characteristics...