91 research outputs found

    Global extent and drivers of mammal population declines in protected areas under illegal hunting pressure

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    Illegal hunting is a persistent problem in many protected areas, but an overview of the extent of this problem and its impact on wildlife is lacking. We reviewed 40 years (1980–2020) of global research to examine the spatial distribution of research and socio-ecological factors influencing population decline within protected areas under illegal hunting pressure. From 81 papers reporting 988 species/site combinations, 294 mammal species were reported to have been illegally hunted from 155 protected areas across 48 countries. Research in illegal hunting has increased substantially during the review period and showed biases towards strictly protected areas and the African continent. Population declines were most frequent in countries with a low human development index, particularly in strict protected areas and for species with a body mass over 100 kg. Our results provide evidence that illegal hunting is most likely to cause declines of large-bodied species in protected areas of resource-poor countries regardless of protected area conservation status. Given the growing pressures of illegal hunting, increased investments in people’s development and additional conservation efforts such as improving anti-poaching strategies and conservation resources in terms of improving funding and personnel directed at this problem are a growing priority

    Heimler Syndrome is Caused by Hypomorphic Mutations in the Peroxisome-Biogenesis Genes PEX1 and PEX6

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    Heimler syndrome (HS) is a rare recessive disorder characterized by sensorineural hearing loss (SNHL), amelogenesis imperfecta, nail abnormalities and occasional or late onset retinal pigmentation. We ascertained eight families with HS, and - using a whole exome sequencing approach - identified biallelic mutations in PEX1 or PEX6 in six of them. Loss of function mutations in both genes are known causes of a spectrum of autosomal recessive peroxisome biogenesis disorders (PBDs), including Zellweger syndrome. PBDs are characterized by leukodystrophy, hypotonia, SNHL, retinopathy, and skeletal, craniofacial, and liver abnormalities. We demonstrate that each HS family has at least one hypomorphic allele that results in extremely mild peroxisomal dysfunction. Although individuals with HS share some subtle clinical features found in PBDs, the overlap is minimal and the diagnosis was not suggested by routine blood and skin fibroblast analyses used to detect PBDs. In conclusion, our findings define Heimler syndrome as a mild PBD, expanding the pleiotropy of mutations in PEX1 and PEX6

    Dynamic global-to-direct irradiance conversion models

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    This paper presents two models for converting hourly global into hourly direct beam irradiance based on a new parameterization of insolation conditions (Perez et al. 1990a). This parameterization does not require any additional input data than those already contained in a global irradiance time series but attempts to better utilize available information. The models are capable of using surface dew-point temperature as an additional input when this is available. The models are derived statistically from a large multiclimatic experimental data base. The two models differ technically but should be functionally equivalent: The first is a correction to the physically based DISC model(Maxwell 1987); the second consists of a set of simple linear relationships. Validation results are presented in reference to three existing models (Maxwell 1987; Erbs et al. 1982; Randall and Whitson 1977). The validation benchmarks consist of root mean square and mean bias errors as well as the ability of the models to recreate the skewness and kurtosis of actual direct irradiance distributions
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