Ionic effects on the membrane potential of hyperpolarizing photoreceptor in scallop retina

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/109976/1/tjp19782751345.pd

Axiomatic Bargaining Theory on Opportunity Assignments

This paper discusses issues of axiomatic bargaining problems over opportunity assignments. The fair arbitrator uses the principle of "equal opportunity" for all players to make the recommendation on re- source allocations. A framework in such a context is developed and several classical solutions to standard bargaining problems are reformulated and axiomatically characterized. Working Paper 06-4

The effects of spatial constraints on the evolution of weighted complex networks

Motivated by the empirical analysis of the air transportation system, we define a network model that includes geographical attributes along with topological and weight (traffic) properties. The introduction of geographical attributes is made by constraining the network in real space. Interestingly, the inclusion of geometrical features induces non-trivial correlations between the weights, the connectivity pattern and the actual spatial distances of vertices. The model also recovers the emergence of anomalous fluctuations in the betweenness-degree correlation function as first observed by Guimer\`a and Amaral [Eur. Phys. J. B {\bf 38}, 381 (2004)]. The presented results suggest that the interplay between weight dynamics and spatial constraints is a key ingredient in order to understand the formation of real-world weighted networks

Coordination changes in liquid tin under shock compression determined using in situ femtosecond x-ray diffraction

Little is known regarding the liquid structure of materials compressed to extreme conditions, and even less is known about liquid structures undergoing rapid compression on nanosecond timescales. Here, we report on liquid structure factor and radial distribution function measurements of tin shock compressed to 84(19) GPa. High-quality, femtosecond x-ray diffraction measurements at the Linac Coherent Light Source were used to extract the liquid diffuse scattering signal. From the radial distribution function, we find that the structural evolution of the liquid with increasing pressure mimics the evolution of the solid phase. With increasing pressure, we find that the liquid structure evolves from a complex structure, with a low coordination number, to a simple liquid structure with a coordination number of 12. We provide a pathway for future experiments to study liquids at elevated pressures using high-energy lasers to shock compress materials beyond the reach of static diamond anvil cell techniques

Identification of a novel heterozygous guanosine monophosphate reductase (GMPR) variant in a patient with a late-onset disorder of mitochondrial DNA maintenance

Autosomal dominant progressive external ophthalmoplegia (adPEO) is a late-onset, Mendelian mitochondrial disorder characterised by paresis of the extraocular muscles, ptosis and skeletal-muscle restricted multiple mitochondrial DNA (mtDNA) deletions. While dominantly-inherited, pathogenic variants in POLG, TWNK and RRM2B are among the most common genetic defects of adPEO, identification of novel candidate genes and the underlying pathomechanisms remain challenging. We report the clinical, genetic and molecular investigations of a patient who presented in the seventh decade of life with PEO. Oxidative histochemistry revealed cytochrome c oxidase deficient fibres and occasional ragged red fibres showing subsarcolemmal mitochondrial accumulation in skeletal muscle, while molecular studies identified the presence of multiple mtDNA deletions. Negative candidate screening of known nuclear genes associated with PEO prompted diagnostic exome sequencing, leading to the prioritisation of a novel heterozygous c.547G > C variant in GMPR (NM_006877.3) encoding guanosine monophosphate reductase, a cytosolic enzyme required for maintaining the cellular balance of adenine and guanine nucleotides. We show that the novel c.547G > C variant causes aberrant splicing, decreased GMPR protein levels in patient skeletal muscle, proliferating and quiescent cells and is associated with subtle changes in nucleotide homeostasis protein levels and evidence of disturbed mtDNA maintenance in skeletal muscle. Despite confirmation of GMPR deficiency, demonstrating marked defects of mtDNA replication or nucleotide homeostasis in patient cells proved challenging. Our study proposes that GMPR is the nineteenth (19th) locus for PEO and highlights the complexities of uncovering disease mechanisms in late-onset PEO phenotypes