Tay-Sachs disease (TSD) is a recessively inherited neurodegenerative disorder caused by mutations in the HEXA gene resulting in β-hexosaminidase A (HEX A) deficiency and neuronal accumulation of GM2 ganglioside. We describe the first patient with Tay-Sachs disease in the Cypriot population, a juvenile case which presented with developmental regression at the age of five. The diagnosis was confi...

In this paper, necessary and sufficient conditions are stated for the Craig-Sakamoto equation det(I − sA − tB) = det(I − sA)det(I − tB) to hold for all scalars s, t ∈ C. Moreover, spectral properties for matrices A and B that satisfy this equation are investigated.

S'Ve PictS Neuter tells her story in simple yet arresting language, giving a very comprehensive des UI~k man ̂ euter> who was her teacher, her colleague, and finally her husband. Po\ver Qp ? .^'m as an extraordinarily dynamic person, with an unshakable belief in the Hany 0r sP'r'1 over matter. His enthusiasm attracted large numbers of people to him, ^ey?te th ? ^ were prepared to withstand the t...

This paper introduces the class of reflected number systems. Members of this class have the adjacency property, i.e. representations for consecutive numbers differ in only one digit and the magnitude of this difference is one. It shows that every number system based on a positional weighting scheme has a reflected analogue. It presents procedures to convert a number system to its reflected anal...

Previous metaphor studies have paid much attention to nominal and predicative metaphors and little attention has been given to adjective metaphors. Although some studies have focused on adjective metaphors, they have only examined how the acceptability of adjective metaphors can be explained by the pairing of adjective modifier’s and head noun’s modalities and little attention has been given to...

This article discusses the difficulties that arose when attempting to specify and design a large scale digital learning environment for Scottish schools. This had a potential user base of about 1 million users and was intended to replace an existing, under-used system. We found that the potential system users were not interested in engaging with the project and that there were immense problems ...

The title compound, C(16)H(16)O(8), is a potent nonspecific phyto-toxin. The crystal structure is the average of two tauto-mers, 5,6,7,9,10-penta-hydr-oxy-2-meth-oxy-7-methyl-1,4,5,6,7,8-hexa-hydro-anthracene-1,4-dione and 1,4,5,6,7-pentahydr-oxy-2-meth--oxy-7-methyl-5,6,7,8,9,10-hexa-hydro-anthracene-9,10-di-one. The cyclo-hexene rings in both tautomers display a half-chair conformation. An ex...