The spectral properties of a rigidified trimethine cyanine dye, Cy3B have been characterised. This probe has excellent fluorescent properties, good water solubility and can be bioconjugated. The emission properties of this fluorophore have also been investigated upon conjugation to an antibody. This study compared the conjugated emission properties of Cy3B with other commercially available fluorophores emitting at similar wavelengths.

Costello syndrome is a rare, autosomal dominant syndrome caused by activating missense mutations in the Harvey rat sarcoma viral oncogene homolog (HRAS), most often p.G12S. Several rare mutations have consistently been associated with a more severe phenotype which is often lethal in infancy. Cause of death is most often respiratory failure with hypertrophic cardiomyopathy playing a significant role in morbidity. Impaired fibroblast elastogenesis is thought to contribute to the Costello phenotype, but reports of histologic evidence of disordered elastogenesis at autopsy are limited. We report a patient with Costello syndrome due to a rare tandem base substitution (c.35_36GC>AA) resulting in the p.G12E missense change. The proband died at age 3 months from respiratory failure, with minimal evidence for cardiomyopathy. Autopsy disclosed pulmonary vascular dysplasia affecting small arteries and veins associated with abnormal elastin distribution in tortuous dilated arteries and veins, with non-uniform wall thickness and semi-obstructive lesions at artery branch points typical of early pulmonary hypertensive vascular disease. Elastic fibers in the dermis were abnormally short and fragmented. This case suggests that disordered elastogenesis in the pulmonary vasculature and undiagnosed (or underdiagnosed) pulmonary hypertension may contribute to morbidity in patients with Costello syndrome.

Abstract:Once a minority language has been introduced to formal schooling, promoters are frequently dismayed by the continuing presence, even dominance, of the major language in the children's school life. In the Basque Autonomous Community (Spain) the regional Government, responsible for primary and secondary schooling, has adopted a series of measures over the past 20 years since the introduction of Basque in its schools alongside Spanish to counter this occurrence. This process has culminated in the creation of an overarching programme for the normalisation of the use of Basque in school by the name of Ulibarri which individual schools can opt into. After describing the context of Basque language revitalisation and the programme's antecedents, the authors offer a detailed picture of the motives, organisation and execution of the Ulibarri programme, together with an evaluation of results, in the belief that this programme with its mix of top-down and bottom-up language planning elements is an innovation of potential interest to other language loyalists worldwide. Efforts to reverse language shift (RLS) towards either Spanish or French have had particular impact in the educational sphere, with Basque becoming a school subject and even teaching medium on an ever-increasing scale throughout the Basque Country in primary, secondary and university teaching. Basque as subject is compulsory in both state and private primary and secondary schools in the BAC and part of neighbouring Navarre, optional (but not always freely available) in the rest of Navarre and in the French Basque Country. Basque-medium teaching is optional throughout the Basque Country, though still difficult to obtain in some areas particularly in southern Navarre and France. Availability of Basque-medium university teaching is largely limited to the BAC. The development of this bilingual educational system is discussed in Stuijt et al. (1998) for the French Basque Country, in Gardner (2000b) for the whole Basque area in Spain and in Gardner (2000a) and Zalbide (1990Zalbide ( , 1999Zalbide ( , 2000 for the BAC. Basque-language immersion education is 4 discussed in Arzamendi and Genesee (1997). From this point on only developments in primary and secondary education in the BAC will be considered.In seeking to establish a bilingual education system throughout the BAC to replace the previously almost entirely monolingual system in Spanish a number of specific questions have been attended to over the last 20 years or so: changes in the law, securing a supply of teachers competent in Basque, the provision of teaching/learning materials in Basque and adding a Basque dimension to the curriculum. The Government shall adopt those measures that will lead to a guarantee of a real possibility, in equality of conditions, of possessing sufficient practical knowledge of Basque at the end of the period of compulsory schooling.To achieve that end the Education Department designed three programmes or models of bilingual teaching:• Spanish-medium teaching with Basque as...

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