Is Multiple System Atrophy a Prion-like Disorder?

Multiple system atrophy (MSA) is a rapidly progressive, fatal neurodegenerative disease of uncertain aetiology that belongs to the family of α-synucleinopathies. It clinically presents with parkinsonism, cerebellar, autonomic, and motor impairment in variable combinations. Pathological hallmarks are fibrillary α-synuclein (αSyn)-rich glial cytoplasmic inclusions (GCIs) mainly involving oligodendroglia and to a lesser extent neurons, inducing a multisystem neurodegeneration, glial activation, and widespread demyelinization. The neuronal αSyn pathology of MSA has molecular properties different from Lewy bodies in Parkinson’s disease (PD), both of which could serve as a pool of αSyn (prion) seeds that could initiate and drive the pathogenesis of synucleinopathies. The molecular cascade leading to the “prion-like” transfer of “strains” of aggregated αSyn contributing to the progression of the disease is poorly understood, while some presented evidence that MSA is a prion disease. However, this hypothesis is difficult to reconcile with postmortem analysis of human brains and the fact that MSA-like pathology was induced by intracerebral inoculation of human MSA brain homogenates only in homozygous mutant 53T mice, without production of disease-specific GCIs, or with replication of MSA prions in primary astrocyte cultures from transgenic mice expressing human αSyn. Whereas recent intrastriatal injection of Lewy body-derived or synthetic human αSyn fibrils induced PD-like pathology including neuronal αSyn aggregates in macaques, no such transmission of αSyn pathology in non-human primates by MSA brain lysate has been reported until now. Given the similarities between αSyn and prions, there is a considerable debate whether they should be referred to as “prions”, “prion-like”, “prionoids”, or something else. Here, the findings supporting the proposed nature of αSyn as a prion and its self-propagation through seeding as well as the transmissibility of neurodegenerative disorders are discussed. The proof of disease causation rests on the concordance of scientific evidence, none of which has provided convincing evidence for the classification of MSA as a prion disease or its human transmission until now.     

Salinity effect on the compaction behaviour,matric suction,stiffness and microstructure of a silty soil

To better understand the salinity effect on the compaction behaviour of soil,standard Proctor compaction test was conducted on soil samples with different salinities.Matric suction and small-strain shear modulus,G_(max),were determined and pore size distribution was also investigated on samples statically compacted at different water contents.Results showed that with the decrease of soil salinity from initial value of 2.1‰(g of salt/kg of dry soil) to zero,the maximum dry density increased and the optimum water content decreased,whereas there was no significant change with the increase of soil salinity from2.1 ‰ to 6.76‰.Interestingly,it was observed that G_(max) also decreased when the soil salinity decreased from initial value of 2.1‰ to zero and kept almost constant when the soil salinity increased from 2.1‰ to6.76‰, for dry samples with similar matric suction and also for samples compacted at optimum state and on wet side whose matric suctions were slightly different due to the difference in remoulded water content.Furthermore,the effect of salinity on compaction behaviour and G_(max) decreased for samples compacted from dry side to wet side.The pore size distribution exhibited bi-modal characteristics with two populations of micro-and macro-pores not only for samples compacted on dry side and at optimum state,but also for those compacted on wet side.Further examination showed that the modal size of micro-pores shifted to lower values and that of macro-pores shifted to higher values for saline soil compared to the soil without salt.     

Analysis of thermal performance of building attached sunspace

The thermal performance of a sunspace attached to a living room located in Amman-Jordan has been investigated. Six configurations that differ by the ratio of glazed surface area to opaque surfaces area are studied. A novel configuration that utilizes an inclined front surface is thermally investigated. The effect of orientation of the sunspace, opaque wall and floor absorption coefficients and number of glass layers on the thermal performance is evaluated. Results show that the sunspace reduces the heating load during the winter, while it creates a serious overheating problem during summer. The contribution of reducing heating requirements increases with increasing the ratio of glazed surface to opaque surface area. Also, the optimal contribution was obtained when the sunspace was oriented to the south. Two passive cooling techniques are proposed and evaluated to overcome the summer overheating problem. Additionally, a passive heating technique is proposed to minimize the thermal losses during winter nights. Internal shading and night ventilation successfully minimize the overheating problem. Employing the three passive techniques with sunspace, results show that as high as 42% reductions in annual heating and cooling load can be achieved. Utilizing an inclined front surface with double layer of glass can further reduce heating and cooling load requirements.     

Synthesis and electrochemical polymerisation of 3′-functionalised terthiophenes: Electrochemical and spectroelectrochemical characterisation

A new terthienyl derivative, 4′-[(2,2′:5′,2″-terthien-3′-yl)methoxy]-2,2′:6′,2″-terpyridine, was synthesised in order to electrogenerate a new conducting polymer as an electrode coating suitable to complex metal ions. The characteristics of the system obtained have been defined through electrochemical and spectroelectrochemical techniques. The physical and chemical properties of 4′-[(2,2′:5′,2″-terthien-3′-yl)methoxy]-2,2′:6′,2″-terpyridine are compared with those of 3′-hydroxymethyl-2,2′:5′,2″-terthiophene, i.e. the corresponding trimer not bearing the terpyridine ligand. Under the same conditions for both monomers, both electrochemical and spectroelectrochemical tests show that 4′-[(2,2′:5′,2″-terthien-3′-yl)methoxy]-2,2′:6′,2″-terpyridine leads to the formation of a narrower molecular weight distribution, with shorter-conjugated polymer chains.