2,463 research outputs found
Structure of Flux Line Lattices with Weak Disorder at Large Length Scales
Dislocation-free decoration images containing up to 80,000 vortices have been
obtained on high quality BiSrCaCuO superconducting
single crystals. The observed flux line lattices are in the random manifold
regime with a roughening exponent of 0.44 for length scales up to 80-100
lattice constants. At larger length scales, the data exhibit nonequilibrium
features that persist for different cooling rates and field histories.Comment: 4 pages, 3 gif images, to appear in PRB rapid communicatio
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Next generation sequencing with copy number variant detection expands the phenotypic spectrum of HSD17B4-deficiency
Background: D-bifunctional protein deficiency, caused by recessive mutations in HSD17B4, is a severe, infantile-onset disorder of peroxisomal fatty acid oxidation. Few affected patients survive past two years of age. Compound heterozygous mutations in HSD17B4 have also been reported in two sisters diagnosed with Perrault syndrome (MIM # 233400), who presented in adolescence with ovarian dysgenesis, hearing loss, and ataxia. Case presentation: An adult male presented with cerebellar ataxia, peripheral neuropathy, hearing loss, and azoospermia. The clinical presentation, in combination with biochemical findings in serum, urine, and muscle biopsy, suggested a mitochondrial disorder. Commercial genetic testing of 18 ataxia and mitochondrial disease genes was negative. Targeted exome sequencing followed by analysis of single nucleotide variants and small insertions/deletions failed to reveal a genetic basis of disease. Application of a computational algorithm to infer copy number variants (CNVs) from exome data revealed a heterozygous 12 kb deletion of exons 10–13 of HSD17B4 that was compounded with a rare missense variant (p.A196V) at a highly conserved residue. Retrospective review of patient records revealed mildly elevated ratios of pristanic:phytanic acid and arachidonic:docosahexaenoic acid, consistent with dysfunctional peroxisomal fatty acid oxidation. Conclusion: Our case expands the phenotypic spectrum of HSD17B4-deficiency, representing the first male case reported with infertility. Furthermore, it points to crosstalk between mitochondria and peroxisomes in HSD17B4-deficiency and Perrault syndrome
A nonlinear model of passive muscle viscosity
The material properties of passive skeletal muscle are critical to proper function and are frequently a target for therapeutic and interventional strategies. Investigations into the passive viscoelasticity of muscle have primarily focused on characterizing the elastic behavior, largely neglecting the viscous component. However, viscosity is a sizeable contributor to muscle stress and extensibility during passive stretch and thus there is a need for characterization of the viscous as well as the elastic components of muscle viscoelasticity. Single mouse muscle fibers were subjected to incremental stress relaxation tests to characterize the dependence of passive muscle stress on time, strain and strain rate. A model was then developed to describe fiber viscoelasticity incorporating the observed nonlinearities. The results of this model were compared with two commonly used linear viscoelastic models in their ability to represent fiber stress relaxation and strain rate sensitivity. The viscous component of mouse muscle fiber stress was not linear as is typically assumed, but rather a more complex function of time, strain and strain rate. The model developed here, which incorporates these nonlinearities, was better able to represent the stress relaxation behavior of fibers under the conditions tested than commonly used models with linear viscosity. It presents a new tool to investigate the changes in muscle viscous stresses with age, injury and disuse
Coulomb Gap and Correlated Vortex Pinning in Superconductors
The positions of columnar pins and magnetic flux lines determined from a
decoration experiment on BSCCO were used to calculate the single--particle
density of states at low temperatures in the Bose glass phase. A wide Coulomb
gap is found, with gap exponent , as a result of the long--range
interaction between the vortices. As a consequence, the variable--range hopping
transport of flux lines is considerably reduced with respect to the
non--interacting case, the effective Mott exponent being enhanced from to for this specific experiment.Comment: 10 pages, Revtex, 4 figures appended as uu-encoded postscript files,
also available as hardcopies from [email protected]
Target specificity among canonical nuclear poly(A) polymerases in plants modulates organ growth and pathogen response
Polyadenylation of pre-mRNAs is critical for efficient nuclear export, stability, and translation of the mature mRNAs, and thus for gene expression. The bulk of pre-mRNAs are processed by canonical nuclear poly(A) polymerase (PAPS). Both vertebrate and higher-plant genomes encode more than one isoform of this enzyme, and these are coexpressed in different tissues. However, in neither case is it known whether the isoforms fulfill different functions or polyadenylate distinct subsets of pre-mRNAs. Here we show that the three canonical nuclear PAPS isoforms in Arabidopsis are functionally specialized owing to their evolutionarily divergent C-terminal domains. A strong loss-of-function mutation in PAPS1 causes a male gametophytic defect, whereas a weak allele leads to reduced leaf growth that results in part from a constitutive pathogen response. By contrast, plants lacking both PAPS2 and PAPS4 function are viable with wild-type leaf growth. Polyadenylation of SMALL AUXIN UP RNA (SAUR) mRNAs depends specifically on PAPS1 function. The resulting reduction in SAUR activity in paps1 mutants contributes to their reduced leaf growth, providing a causal link between polyadenylation of specific pre-mRNAs by a particular PAPS isoform and plant growth. This suggests the existence of an additional layer of regulation in plant and possibly vertebrate gene expression, whereby the relative activities of canonical nuclear PAPS isoforms control de novo synthesized poly(A) tail length and hence expression of specific subsets of mRNAs
Lamb Shift of 3P and 4P states and the determination of
The fine structure interval of P states in hydrogenlike systems can be
determined theoretically with high precision, because the energy levels of P
states are only slightly influenced by the structure of the nucleus. Therefore
a measurement of the fine structure may serve as an excellent test of QED in
bound systems or alternatively as a means of determining the fine structure
constant with very high precision. In this paper an improved analytic
calculation of higher-order binding corrections to the one-loop self energy of
3P and 4P states in hydrogen-like systems with low nuclear charge number is
presented. A comparison of the analytic results to the extrapolated numerical
data for high ions serves as an independent test of the analytic
evaluation. New theoretical values for the Lamb shift of the P states and for
the fine structure splittings are given.Comment: 33 pages, LaTeX, 4 tables, 4 figure
Coherent Single Charge Transport in Molecular-Scale Silicon Nanowire Transistors
We report low-temperature electrical transport studies of molecule-scale
silicon nanowires. Individual nanowires exhibit well-defined Coulomb blockade
oscillations characteristic of charge addition to a single nanostructure with
length scales up to at least 400 nm. Further studies demonstrate coherent
charge transport through discrete single particle quantum levels extending the
whole device, and show that the ground state spin configuration follows the
Lieb-Mattis theorem. In addition, depletion of the nanowires suggests that
phase coherent single-dot characteristics are accessible in a regime where
correlations are strong.Comment: 4 pages and 4 figure
Unintentional high density p-type modulation doping of a GaAs/AlAs core-multi-shell nanowire
Achieving significant doping in GaAs/AlAs core/shell nanowires (NWs) is of
considerable technological importance but remains a challenge due to the
amphoteric behavior of the dopant atoms. Here we show that placing a narrow
GaAs quantum well in the AlAs shell effectively getters residual carbon
acceptors leading to an \emph{unintentional} p-type doping. Magneto-optical
studies of such a GaAs/AlAs core multi-shell NW reveal quantum confined
emission. Theoretical calculations of NW electronic structure confirm quantum
confinement of carriers at the core/shell interface due to the presence of
ionized carbon acceptors in the 1~nm GaAs layer in the shell.
Micro-photoluminescence in high magnetic field shows a clear signature of
avoided crossings of the Landau level emission line with the Landau
level TO phonon replica. The coupling is caused by the resonant hole-phonon
interaction, which points to a large 2D hole density in the structure.Comment: just published in Nano Letters
(http://pubs.acs.org/doi/full/10.1021/nl500818k
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