Erratum: Dominant-negative mutations in human IL6ST underlie hyper-IgE syndrome (J. Exp. Med. (2020) 217:6 Doi:10.1084/jem.20191804)

The authors regret that in the original version of Table S1, the column for patient 12 was mistakenly duplicated in the column for patient 8. The online Table S1 PDF has been corrected. The error appears only in PDFs downloaded before June 4, 2020

Dominant-negative mutations in human IL6ST underlie hyper-IgE syndrome

Autosomal dominant hyper-IgE syndrome (AD-HIES) is typically caused by dominant-negative (DN) STAT3 mutations. Patients suffer from cold staphylococcal lesions and mucocutaneous candidiasis, severe allergy, and skeletal abnormalities. We report 12 patients from 8 unrelated kindreds with AD-HIES due to DN IL6ST mutations. We identified seven different truncating mutations, one of which was recurrent. The mutant alleles encode GP130 receptors bearing the transmembrane domain but lacking both the recycling motif and all four STAT3-recruiting tyrosine residues. Upon overexpression, the mutant proteins accumulate at the cell surface and are loss of function and DN for cellular responses to IL-6, IL-11, LIF, and OSM. Moreover, the patients’ heterozygous leukocytes and fibroblasts respond poorly to IL-6 and IL-11. Consistently, patients with STAT3 and IL6ST mutations display infectious and allergic manifestations of IL-6R deficiency, and some of the skeletal abnormalities of IL-11R deficiency. DN STAT3 and IL6ST mutations thus appear to underlie clinical phenocopies through impairment of the IL-6 and IL-11 response pathways

Smallholder dairy production and markets: a comparison of production systems in Zambia, Kenya and Sri Lanka

Three smallholder dairy production systems in Zambia, Sri Lanka and Kenya are analysed and compared. The focus is on the relationships between the animal production system, the farm household system, and the institutional environment. Attention is given to the valuation of marketed and non-marketed products and the intangible benefits of livestock in insurance, financing and status display. The comprehensive and comparative analysis of the production systems shows the direct relationship between type and intensity of dairy production and the presence or absence of markets for milk and other products, services and employment. The generally unobserved income components resulting from products other than milk and from the intangible benefits prove to be a substantial proportion of the total income in all three systems. The comprehensive perspective on the dairy production system results in policy suggestions that include institutional linkages

Characterization of the spatial distribution of farming systems in the Kenyan Highlands

Land cover change maps are not sufficient to identify subtle changes in land use and farming systems. This paper describes a method that is developed to identify the spatial distribution of farming system types without the need to extensively map all farming systems across a large region. Moreover, it explains differences between farming systems based on spatial variation in environmental and socio-economic conditions. In the study area farming systems were characterized and classified based on the criteria area under cultivation of both food and cash crops, milk production and the usage of fertilizers. Logit models were fitted to explain differences in farming system using location factors and household characteristics. A model based on an integrated set of household and location factors best described the diversity of farming systems across the region. However, the location factors alone also described a larger part of the diversity. The spatial variation in location factors and household characteristics were used to determine the likelihood of occurrence of the different farming systems across the study area. By assigning the farming system to a location that best fits the local conditions based on the logit model a regional level farming systems map for the Kenyan Highlands was created. The methodology provides a tool of analyzing spatial variation in farming systems complementary to the analysis of farming systems at the household level and gives insight in the spatial determinants of these systems

Location and uptake: integrated household and GIS analysis of technology adoption and land use, with application to smallholder dairy farms in Kenya

GIS-derived measures of location and space have increasingly been used in models of land use and ecology. However, they have made few inroads into the literature on technology adoption in developing countries, which continues to rely mainly on survey-derived information. Location, with all its dimensions of market access, demographics and agro-climate, nevertheless remains key to understanding potential for technology use. The measures oflocation typically used in the adoption literature, such as locational dummy variables that proxy a range of locational factors, now appear relatively crude given the increased availability of more explicit GIS-derived measures. This paper attempts to demonstrate the usefulness of integrating GIS-measures into analysis of technology uptake, for better differentiating and understanding locational effects. A set of GIS-derived measures of market access and agro-climate are included in a standard household model of technology uptake, applied to smallholder dairy farms in Kenya, using a sample of 3330 geo-referenced farm households. The three technologies examined are keeping of dairy cattle, planting of specialised fodder, and use of concentrate feed. Logit estimations are conducted that significantly differentiate effects of individual household characteristics from those related to location. The predicted values of the locational variables are then used to make spatial predictions of technology potential. Comparisons are made with estimations based only on survey data, which demonstrate that while overall explanatory power may not improve with GIS-derived variables, the latter yield more practical interpretations, which is further demonstrated through predictions of technology uptake change with a shift in infrastructure policy. Although requiring large geo-referenced data sets and high resolution GIS layers, the methodology demonstrates the potential to better unravel the multiple effects of location on farmer decisions on technology and land use. © 2002 Elsevier Science B.V. All rights reserved