Progression of myopathology in Kearns-Sayre syndrome

We report on the progression of myopathology by comparing two biopsies from a patient with a Kearns-Sayre-Syndrome. The first biopsy was taken in 1979 and showed 10% ragged-red fibers. Myopathic changes were slight including internal nuclei and fiber splitting in 10% of the fibers. Electron microscopy revealed typical mitochondrial abnormalities with regard to number and shape. In 1989 a second biopsy was performed for an extended analysis of mitochondrial DNA. This time less than 5% of all fibers were ragged-red. Severe myopathic changes could be detected which so far has rarely been reported in mitochondrial cytopathy

Exploring polymer/nanoparticle hybrid solar cells in tandem architecture

Tandem solar cells offer the possibility to significantly enhance solar cell performance through harvesting a broader part of the solar spectrum by using complementary absorbing materials. We report on tandem solar cells, with at least one polymer/nanoparticle hybrid layer as absorber material, in which the nanoparticles are prepared in situ by thermal decomposition of metal xanthates directly in the polymer matrix. In a first series, we investigated a hybrid-organic tandem solar cell, with a hybrid solar cell consisting of the silafluorene containing low band gap polymer PSiF-DBT and copper indium sulphide (CIS) nanoparticles as the bottom cell, and a low band gap polymer (PTB7)/fullerene derivative (PC61BM) organic solar cell as the top cell in order to study different recombination layers. Tandem devices with open circuit voltages nearly reaching the sum of the individual cells have been realised. The short circuit current is equal to the value of the hybrid single cell and a fill factor above 50% is obtained, leading to power conversion efficiencies of about 4.1%. Furthermore, the first results on hybrid-hybrid tandem solar cells consisting of two PSiF-DBT/CIS solar cells are presented. Although the preparation of these double hybrid devices is challenging because of the necessity of two thermal annealing steps, the resulting multilayer stack reveals smooth and homogeneous layers with sharp interfaces. The first working hybrid-hybrid tandem solar cells still exhibited 81% of the sum of the open circuit voltages of the single junction solar cells. © 2013 The Royal Society of Chemistry

A Fermi Surface Model for Large Supersymmetric AdS_5 Black Holes

We identify a large family of 1/16 BPS operators in N=4 SYM that qualitatively reproduce the relations between charge, angular momentum and entropy in regular supersymmetric AdS_5 black holes when the main contribution to their masses is given by their angular momentum.Comment: 32 pages, 6 figures, LaTeX uses JHEP3 class; ver 2- added acknowledgment, minor change

The crystal structure of Fe₄S₄ quinolinate synthase unravels an enzymatic dehydration mechanism that uses tyrosine and a hydrolase-type triad.

International audienceQuinolinate synthase (NadA) is a Fe4S4 cluster-containing dehydrating enzyme involved in the synthesis of quinolinic acid (QA), the universal precursor of the essential nicotinamide adenine dinucleotide (NAD) coenzyme. A previously determined apo NadA crystal structure revealed the binding of one substrate analog, providing partial mechanistic information. Here, we report on the holo X-ray structure of NadA. The presence of the Fe4S4 cluster generates an internal tunnel and a cavity in which we have docked the last precursor to be dehydrated to form QA. We find that the only suitably placed residue to initiate this process is the conserved Tyr21. Furthermore, Tyr21 is close to a conserved Thr-His-Glu triad reminiscent of those found in proteases and other hydrolases. Our mutagenesis data show that all of these residues are essential for activity and strongly suggest that Tyr21 deprotonation, to form the reactive nucleophilic phenoxide anion, is mediated by the triad. NadA displays a dehydration mechanism significantly different from the one found in archetypical dehydratases such as aconitase, which use a serine residue deprotonated by an oxyanion hole. The X-ray structure of NadA will help us unveil its catalytic mechanism, the last step in the understanding of NAD biosynthesis

On Field Theory Thermalization from Gravitational Collapse

Motivated by its field theory interpretation, we study gravitational collapse of a minimally coupled massless scalar field in Einstein gravity with a negative cosmological constant. After demonstrating the accuracy of the numerical algorithm for the questions we are interested in, we investigate various aspects of the apparent horizon formation. In particular, we study the time and radius of the apparent horizon formed as functions of the initial Gaussian profile for the scalar field. We comment on several aspects of the dual field theory picture.Comment: 31 pages, 17 figures; V2 Some figures corrected, minor revision. arXiv admin note: substantial text overlap with arXiv:1106.233

Ruthenium polypyridyl complexes and their modes of interaction with DNA : is there a correlation between these interactions and the antitumor activity of the compounds?

Various interaction modes between a group of six ruthenium polypyridyl complexes and DNA have been studied using a number of spectroscopic techniques. Five mononuclear species were selected with formula [Ru(tpy) L1L2](2-n)?, and one closely related dinuclear cation of formula [{Ru(apy)(tpy)}2{l-H2N(CH2)6NH2}]4?. The ligand tpy is 2,20:60,200-terpyridine and the ligand L1 is a bidentate ligand, namely, apy (2,20-azobispyridine), 2-phenylazopyridine, or 2-phenylpyridinylmethylene amine. The ligand L2 is a labile monodentate ligand, being Cl-, H2O, or CH3CN. All six species containing a labile L2 were found to be able to coordinate to the DNA model base 9-ethylguanine by 1H NMR and mass spectrometry. The dinuclear cationic species, which has no positions available for coordination to a DNA base, was studied for comparison purposes. The interactions between a selection of four representative complexes and calf-thymus DNA were studied by circular and linear dichroism. To explore a possible relation between DNA-binding ability and toxicity, all compounds were screened for anticancer activity in a variety of cancer cell lines, showing in some cases an activity which is comparable to that of cisplatin. Comparison of the details of the compound structures, their DNA binding, and their toxicity allows the exploration of structure–activity relationships that might be used to guide optimization of the activity of agents of this class of compounds

Bulk Damage Effects in Irradiated Silicon Detectors due to Clustered Divacancies

High resistivity silicon particle detectors will be used extensively in experiments at the future CERN Large Hadron Collider where the enormous particle fluences give rise to significant atomic displacement damage. A model has been developed to estimate the evolution of defect concentrations during irradiation and their electrical behaviour according to Shockley-Read-Hall (SRH) semiconductor statistics. The observed increases in leakage current and doping concentration changes can be described well after gamma irradiation but less well after fast neutron irradiation. A possible non-SRH mechanism is considered, based on the hypothesis of charge transfer between clustered divacancy defects in neutron damaged silicon detectors. This leads to a large enhancement over the SRH prediction for V2 acceptor state occupancy and carrier generation rate which may resolve the discrepancy