Requirements analysis for decision-support system design: evidence from the automotive industry

The purpose of this paper is to outline the requirements analysis that was carried out to support the development of a system that allows engineers to view real-time data integrated from multiple silos such as Product Lifecycle Management (PLM) and Warranty systems, in a single and visual environment. The outcome of this study provides a clear understanding of how engineers working in different phases of the product-lifecycle could utilise such information to improve the decision making process and as a result design better products. This study uses data collected via in-depth semi-structured interviews and workshops that includes people working in various roles within the automotive sector. In order to demonstrate the applicability this approach, SysML diagrams are also provided

Mutations in CTNNA1 cause butterfly-shaped pigment dystrophy and perturbed retinal pigment epithelium integrity

Butterfly-shaped pigment dystrophy is an eye disease characterized by lesions in the macula that can resemble the wings of a butterfly. Here we report the identification of heterozygous missense mutations in the CTNNA1 gene (encoding alpha-catenin 1) in three families with butterfly-shaped pigment dystrophy. In addition, we identified a Ctnna1 missense mutation in a chemically induced mouse mutant, tvrm5. Parallel clinical phenotypes were observed in the retinal pigment epithelium (RPE) of individuals with butterfly-shaped pigment dystrophy and in tvrm5 mice, including pigmentary abnormalities, focal thickening and elevated lesions, and decreased light-activated responses. Morphological studies in tvrm5 mice demonstrated increased cell shedding and the presence of large multinucleated RPE cells, suggesting defects in intercellular adhesion and cytokinesis. This study identifies CTNNA1 gene variants as a cause of macular dystrophy, indicates that CTNNA1 is involved in maintaining RPE integrity and suggests that other components that participate in intercellular adhesion may be implicated in macular disease