Hereditary sensory neuropathy type 1 (HSN1, MIM 162400; ref. 1) genetically maps to human chromosome 9q22 (refs. 2-4). We report here that the gene encoding a subunit of serine palmitoyltransferase is located within the HSN1 locus, expressed in dorsal root ganglia (DRG) and mutated in HSN1.

Germination and growth of spores of butyric acid bacteria (BAB) may cause severe defects in semihard cheeses. Silage is the main source of BAB spores in cheese milk. The objectives of the study were to determine the significance of grass silages and corn silages as sources of BAB spores and to investigate the relationships between high concentrations of BAB spores in corn silage and aerobic deterioration. In the first survey, samples were taken from various locations in silos containing grass and corn silages and from mixed silages in the ration offered to the cows on 21 farms. We demonstrated that the quantity of BAB spores consumed by cows was determined by a small fraction of silage with a high concentration of spores (above 5 log10 BAB/g). High concentrations were most often found in corn silage within areas with visible molds (69% of the samples). Areas with visible molds in grass silage and surface layers of corn silage contained, respectively, 21 and 19% of the cases of concentrations above 5 log10 BAB spores/g. Based on these results, we concluded that currently in the Netherlands, corn silage is a more important source of BAB than is grass silage. In a second survey, 8 corn silages were divided into 16 sections and each section was studied in detail. High concentrations of BAB spores were found in only the top 50 cm of these 8 silages. Elevated concentrations of BAB spores were associated with different signs of aerobic deterioration. In 13% of the sections in corn silage with more than 5 log10 yeasts and molds/g, more than 5 log10 BAB spores/g were found. Sections with a temperature of more than 5 degrees C above ambient temperature contained, in 21% of the cases, more than 5 log10 BAB spores/g. Concentrations above 5 log10 BAB spores/g were measured in 50% of the sections with a pH above 4.4. All sections with a pH above 4.4 also showed a temperature that was more than 5 degrees C above ambient temperature and a concentration of yeasts and molds above 5 log10 cfu/g. Based on these results, we postulated that high concentrations of BAB spores in corn silage are the result of oxygen penetration into the silage, resulting in aerobic deterioration and the formation of anaerobic niches with an increased pH just below the surface. Growth of BAB in these anaerobic niches with an increased pH caused the locally high concentrations of BAB in corn silage.

No abstract

The authors examine the social constructionist nature of solution-focused therapy. The therapy's components are illustrated through the presentation of a first-session conversation between Insoo Kim Berg as therapist and a 19-year-old mother, who states that she is “stressed out and depressed.” As the conversation develops, the young mother's sense of herself changes and the integration of the therapy's components become apparent. Next, existing outcome research is reviewed. Although descriptive in nature and limited in scope, research suggests positive outcomes for diverse clients and presenting problems. Finally, several connections are made between solution-focused procedures and social constructionist theory: the social construction of reality, language as the medium and substance of meaning, client change through the construction of new meanings, the client as expert, taking a collaborative stance, reflexivity, drawing on client strengths, and solutions as co-constructions.

No abstract