Rapid degradation of mutant SLC25A46 by the ubiquitin-proteasome system results in MFN1/2-mediated hyperfusion of mitochondria.

SCL25A46 is a mitochondrial carrier protein that surprisingly localizes to the outer membrane and is distantly related to Ugo1. Here we show that a subset of SLC25A46 interacts with mitochondrial dynamics components and the MICOS complex. Decreased expression of SLC25A46 results in increased stability and oligomerization of MFN1 and MFN2 on mitochondria, promoting mitochondrial hyperfusion. A mutation at L341P causes rapid degradation of SLC25A46, which manifests as a rare disease, pontocerebellar hypoplasia. The E3 ubiquitin ligases MULAN and MARCH5 coordinate ubiquitylation of SLC25A46 L341P, leading to degradation by organized activities of P97 and the proteasome. Whereas outer mitochondrial membrane-associated degradation is typically associated with apoptosis or a specialized type of autophagy termed mitophagy, SLC25A46 degradation operates independently of activation of outer membrane stress pathways. Thus SLC25A46 is a new component in mitochondrial dynamics that serves as a regulator for MFN1/2 oligomerization. Moreover, SLC25A46 is selectively degraded from the outer membrane independently of mitophagy and apoptosis, providing a framework for mechanistic studies in the proteolysis of outer membrane proteins

Experimental study of electric breakdowns in liquid argon at centimeter scale

In this paper we present results on measurements of the dielectric strength of liquid argon near its boiling point and cathode-anode distances in the range of 0.1 mm to 40 mm with spherical cathode and plane anode. We show that at such distances the applied electric field at which breakdowns occur is as low as 40 kV/cm. Flash-overs across the ribbed dielectric of the high voltage feed-through are observed for a length of 300 mm starting from a voltage of 55 kV. These results contribute to set reference for the breakdown-free design of ionization detectors, such as Liquid Argon Time Projection Chambers (LAr TPC)

On the Electric Breakdown in Liquid Argon at Centimeter Scale

We present a study on the dependence of electric breakdown discharge properties on electrode geometry and the breakdown field in liquid argon near its boiling point. The measurements were performed with a spherical cathode and a planar anode at distances ranging from 0.1 mm to 10.0 mm. A detailed study of the time evolution of the breakdown volt-ampere characteristics was performed for the first time. It revealed a slow streamer development phase in the discharge. The results of a spectroscopic study of the visible light emission of the breakdowns complement the measurements. The light emission from the initial phase of the discharge is attributed to electro-luminescence of liquid argon following a current of drifting electrons. These results contribute to set benchmarks for breakdown-safe design of ionization detectors, such as Liquid Argon Time Projection Chambers (LAr TPC).Comment: Minor revision according to editor report. 17 pages, 15 figures, 2 tables. Turboencabulato

A method to suppress dielectric breakdowns in liquid argon ionization detectors for cathode to ground distances of several millimeters

We present a method to reach electric field intensity as high as 400 kV/cm in liquid argon for cathode-ground distances of several millimeters. This can be achieved by suppressing field emission from the cathode, overcoming limitations that we reported earlier

Measurement of the drift field in the ARGONTUBE LAr TPC with 266~nm pulsed laser beams

ARGONTUBE is a liquid argon time projection chamber (LAr TPC) with a drift field generated in-situ by a Greinacher voltage multiplier circuit. We present results on the measurement of the drift-field distribution inside ARGONTUBE using straight ionization tracks generated by an intense UV laser beam. Our analysis is based on a simplified model of the charging of a multi-stage Greinacher circuit to describe the voltages on the field cage rings

GeneXplorer: an interactive web application for microarray data visualization and analysis

BACKGROUND: When publishing large-scale microarray datasets, it is of great value to create supplemental websites where either the full data, or selected subsets corresponding to figures within the paper, can be browsed. We set out to create a CGI application containing many of the features of some of the existing standalone software for the visualization of clustered microarray data. RESULTS: We present GeneXplorer, a web application for interactive microarray data visualization and analysis in a web environment. GeneXplorer allows users to browse a microarray dataset in an intuitive fashion. It provides simple access to microarray data over the Internet and uses only HTML and JavaScript to display graphic and annotation information. It provides radar and zoom views of the data, allows display of the nearest neighbors to a gene expression vector based on their Pearson correlations and provides the ability to search gene annotation fields. CONCLUSIONS: The software is released under the permissive MIT Open Source license, and the complete documentation and the entire source code are freely available for download from CPAN

Phase Transition Study of Superconducting Microstructures

The presented results are part of a feasibility study of superheated superconducting microstructure detectors. The microstructures (dots) were fabricated using thin film patterning techniques with diameters ranging from $50\mu$m up to $500\mu$m and thickness of $1\mu$m. We used arrays and single dots to study the dynamics of the superheating and supercooling phase transitions in a magnetic field parallel to the dot surface. The phase transi- tions were produced by either varying the applied magnetic field strength at a constant temperature or changing the bath temperature at a constant field. Preliminary results on the dynamics of the phase transitions of arrays and single indium dots will be reported.Comment: 7pages in LaTex format, five figures available upon request by [email protected], preprint Bu-He 93/

Renormalization Group and Universality

It is argued that universality is severely limited for models with multiple fixed points. As a demonstration the renormalization group equations are presented for the potential and the wave function renormalization constants in the $O(N)$ scalar field theory. Our equations are superior compared with the usual approach which retains only the contributions that are non-vanishing in the ultraviolet regime. We find an indication for the existence of relevant operators at the infrared fixed point, contrary to common expectations. This result makes the sufficiency of using only renormalizable coupling constants in parametrizing the long distance phenomena questionable.Comment: 32pp in plain tex; revised version to appear in PR

Quantum-classical crossover in electrodynamics

A classical field theory is proposed for the electric current and the electromagnetic field interpolating between microscopic and macroscopic domains. It represents a generalization of the density functional for the dynamics of the current and the electromagnetic field in the quantum side of the crossover and reproduces standard classical electrodynamics on the other side. The effective action derived in the closed time path formalism and the equations of motion follow from the variational principle. The polarization of the Dirac-see can be taken into account in the quadratic approximation of the action by the introduction of the deplacement field strengths as in conventional classical electrodynamics. Decoherence appears naturally as a simple one-loop effect in this formalism. It is argued that the radiation time arrow is generated from the quantum boundary conditions in time by decoherence at the quantum-classical crossover and the Abraham-Lorentz force arises from the accelerating charge or from other charges in the macroscopic or the microscopic side, respectively. The functional form of quantum renormalization group, the generalization of the renormalization group method for the density matrix, is proposed to follow the scale dependence through the quantum-classical crossover in a systematical manner.Comment: new references added, few sign errors fixed, to appear in Physical Review