Bovine ceroid-lipofuscinosis: pathology of blindness

Five Devon cattle with suspected ceroid-lipofuscinosis and aged between 19 and 39 months of age were humanely slaughtered and subjected to post-mortem examination. There was severe atrophy of the cerebrum, particularly of the occipital cortex. Microscopy also showed severe atrophy of the retina with complete loss of photoreceptor cells, even in the youngest animal examined. Histopathologically the disease was characterised by accumulation of a fluorescent lipopigment in neurones, including those of the retina and a severe astrocytosis. The disease, which is characterised by the accumulation of subunit c of mitochondrial ATP synthase, is similar to that extensively described in South Hampshire sheep except that the retinal lesions were more severe. In contrast, tremors were not noted in the cattle. The clinical history and similarity to the disease in sheep and other species indicated inheritance was as an autosomal recessive trait.     

Congenital arthrogryposis: an inherited limb deformity in pedigree Suffolk lambs

Thirty of 52 pedigree Suffolk lambs (58 per cent) were born with arthrogryposis characterised by bilateral flexion rigidity of the metacarpophalangeal and carpal joints. The recent introduction of a breeding ram was identified as the only significant risk factor in the flock, and embryo transfer was used to test the hypothesis that the arthrogryposis was an inherited disorder associated with the introduction of this ram. Two adult ewes that had previously produced lambs with arthrogryposis by the ram and four of its affected daughters were available as donors, and 20 crossbred ewes were used as recipients. Ten Suffolk-crossed ewes that had no known familial relationship with the ram were also mated by the ram as controls and they produced 10 normal lambs. Following embryo transfer, 19 lambs were born, of which seven were stillborn; arthrogryposis was evident in 10 of the 12 live lambs. Analysis of the data suggested that in the population under study, arthrogryposis was inherited as an autosomal recessive condition.     

Molecular heterogeneity for bovine α-mannosidosis: PCR based assays for detection of breed-specific mutations

tests, based on the polymerase chain reaction ( ), were developed for the detection of two breed-specific mutations responsible for the autosomal recessive disorder bovine α-mannosidosis. The tests involve separate amplification of two exons of the lysosomal α-mannosidase gene followed by restriction enzyme digestion of the amplicons. We demonstrate that one of the mutations, the 662G→A transition, is responsible for α-mannosidosis in Galloway cattle. The other mutation, the 961T→C transition, is uniquely associated with α-mannosidosis in Angus, Murray Grey and Brangus cattle from Australia. The 961T→C mutation was also detected in Red Angus cattle exported from Canada to Australia as embryos. All 39 animals classified as heterozygotes on the basis of biochemical assays were heterozygous for one of the two mutations. None of 102 animals classified as homozygous-normal on the basis of biochemical assays possessed the mutations. Our results indicate that the two breed-specific mutations may have arisen in Scotland and by the export of animals and germplasm disseminated to America, New Zealand and Australia.     

Acanthocephalan infection and sparganosis in a green tree snake (Dendrelaphis punctulata)

Acanthocephalan and spargana parasites were identified within a body wall mass during exploratory surgery in a wild green tree snake. Acanthocephalan parasites have not previously been reported in this species. Surgical excision, the treatment of choice, could not be achieved because of the extensive infiltration of the coelomic cavity.     

Prenatal occurrence and mode of inheritance of neuraxial oedema in Poll Hereford calves

Breeding experiments were conducted in 1981, 1982 and 1983 using bulls and cows that were obligatory heterozygotes for neuraxial oedema. Fifty-six calves were born in the herd, 14 of which were diagnosed as cases of neuraxial oedema. The mean gestation length for affected calves born in 1983 was nine days shorter than that for unaffected calves. Observations were made of the birth of three affected calves. In one calf spasms of the forelimbs were observed during an unassisted delivery, and in this and two others, typical clinical signs were observed within two minutes of birth. Radiological and, or, pathological examination of each of the eight affected calves born in 1983 revealed lesions in the coxae, some of which predated parturition. These observations are consistent with the calves being affected with neuraxial oedema while in utero.     

Probing the Local Effects of Magnetic Impurities on Superconductivity

The local effects of isolated magnetic adatoms on the electronic properties of the surface of a superconductor were studied with a low-temperature scanning tunneling microscope. Tunneling spectra obtained near magnetic adsorbates reveal the presence of excitations within the superconductor's energy gap that can be detected over a few atomic diameters around the impurity at the surface. These excitations are locally asymmetric with respect to tunneling of electrons and holes. A model calculation based on the Bogoliubov-de Gennes equations can be used to understand the details of the local tunneling spectra.     

Spontaneous formation of macroscopic chiral domains in a fluid smectic phase of achiral molecules

A smectic liquid-crystal phase made from achiral molecules with bent cores was found to have fluid layers that exhibit two spontaneous symmetry-breaking instabilities: polar molecular orientational ordering about the layer normal and molecular tilt. These instabilities combine to form a chiral layer structure with a handedness that depends on the sign of the tilt. The bulk states are either antiferroelectric-racemic, with the layer polar direction and handedness alternating in sign from layer to layer, or antiferroelectric-chiral, which is of uniform layer handedness. Both states exhibit an electric field-induced transition from antiferroelectric to ferroelectric.     

Organic Glasses: A New Class of Photorefractive Materials

The performance of amorphous organic photorefractive (PR) materials in applications such as optical data storage is generally limited by the concentration of active molecules (chromophores) that can be incorporated into the host without forming a crystalline material with poor optical quality. In polymeric PR systems described previously, performance has been limited by the necessity of devoting a large fraction of the material to inert polymer and plasticizing components in order to ensure compositional stability. A new class of organic PR materials composed of multifunctional glass-forming organic chromophores is described that have long-term stability and greatly improved PR properties.     

Flow and storage in groundwater systems

The dynamic nature of groundwater is not readily apparent, except where discharge is focused at springs or where recharge enters sinkholes. Yet groundwater flow and storage are continually changing in response to human and climatic stresses. Wise development of groundwater resources requires a more complete understanding of these changes in flow and storage and of their effects on the terrestrial environment and on numerous surface-water features and their biota.