8,290 research outputs found
Vibration of a circular cylindrical elastic tank, partially filled with an incompressible fluid, undergoing an axial acceleration composed of a uniform and a periodic component technical memorandum no. 102
Forced vibration of circular cylindrical elastic shell partially filled with incompressible liquid and initially at rest in uniform gravitational fiel
The clinical spectrum of SMA-PME and in vitro normalization of its cellular ceramide profile
OBJECTIVE: The objectives of this study were to define the clinical and biochemical spectrum of spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME) and to determine if aberrant cellular ceramide accumulation could be normalized by enzyme replacement.
METHODS: Clinical features of 6 patients with SMA-PME were assessed by retrospective chart review, and a literature review of 24 previously published cases was performed. Leukocyte enzyme activity of acid ceramidase was assessed with a fluorescence-based assay. Skin fibroblast ceramide content and was assessed by high performance liquid chromatography, electrospray ionization tandem mass spectroscopy. Enzyme replacement was assessed using recombinant human acid ceramidase (rhAC) in vitro.
RESULTS: The six new patients showed the hallmark features of SMA-PME, with variable initial symptom and age of onset. Five of six patients carried at least one of the recurrent SMA-PME variants observed in two specific codons of ASAH1. A review of 30 total cases revealed that patients who were homozygous for the most common c.125C \u3e T variant presented in the first decade of life with limb-girdle weakness as the initial symptom. Sensorineural hearing loss was associated with the c.456A \u3e C variant. Leukocyte acid ceramidase activity varied from 4.1%-13.1% of controls. Ceramide species in fibroblasts were detected and total cellular ceramide content was elevated by 2 to 9-fold compared to controls. Treatment with rhAC normalized ceramide profiles in cultured fibroblasts to control levels within 48 h.
INTERPRETATION: This study details the genotype-phenotype correlations observed in SMA-PME and shows the impact of rhAC to correct the abnormal cellular ceramide profile in cells
Exact Zeros of the Partition Function for a Continuum System with Double Gaussian Peaks
We calculate the exact zeros of the partition function for a continuum system
where the probability distribution for the order parameter is given by two
asymmetric Gaussian peaks. When the positions of the two peaks coincide, the
two separate loci of zeros which used to give first-order transition touch each
other, with density of zeros vanishing at the contact point on the positive
real axis. Instead of the second-order transition of Ehrenfast classification
as one might naively expect, one finds a critical behavior in this limit.Comment: 13 pages, 6 figures, revtex, minor changes in fig.2, to be published
in Physical Review
Recurrence interval analysis of high-frequency financial returns and its application to risk estimation
We investigate the probability distributions of the recurrence intervals
between consecutive 1-min returns above a positive threshold or
below a negative threshold of two indices and 20 individual stocks in
China's stock market. The distributions of recurrence intervals for positive
and negative thresholds are symmetric, and display power-law tails tested by
three goodness-of-fit measures including the Kolmogorov-Smirnov (KS) statistic,
the weighted KS statistic and the Cram\'er-von Mises criterion. Both long-term
and shot-term memory effects are observed in the recurrence intervals for
positive and negative thresholds . We further apply the recurrence interval
analysis to the risk estimation for the Chinese stock markets based on the
probability , Value-at-Risk (VaR) analysis and VaR analysis
conditioned on preceding recurrence intervals.Comment: 17 pages, 10 figures, 1 tabl
Large scale emergent properties of an autocatalytic reaction-diffusion model subject to noise
The non-equilibrium dynamic fluctuations of a stochastic version of the
Gray-Scott (GS) model are studied analytically in leading order in perturbation
theory by means of the dynamic renormalization group. There is an attracting
stable fixed point at one-loop order, and the asymptotic scaling of the
correlation functions is predicted for both spatial and temporally correlated
noise sources. New effective three-body reaction terms, not present in the
original GS model, are induced by the combined interplay of the fluctuations
and nonlinearities.Comment: 13 pages, 2 figure
Spitzer Observations of the North Ecliptic Pole
We present a photometric catalog for Spitzer Space Telescope warm mission
observations of the North Ecliptic Pole (NEP; centered at , ). The observations are
conducted with IRAC in 3.6 m and 4.5 m bands over an area of 7.04
deg reaching 1 depths of 1.29 Jy and 0.79 Jy in the 3.6
m and 4.5 m bands respectively. The photometric catalog contains
380,858 sources with 3.6 m and 4.5 m band photometry over the
full-depth NEP mosaic. Point source completeness simulations show that the
catalog is 80% complete down to 19.7 AB. The accompanying catalog can be
utilized in constraining the physical properties of extra-galactic objects,
studying the AGN population, measuring the infrared colors of stellar objects,
and studying the extra-galactic infrared background light.Comment: 10 pages, 11 figures and 3 tables. Accepted to the ApJ
Environmental dependence of 8 μm luminosity functions of galaxies at z ~ 0.8: Comparison between RXJ1716.4+6708 and the AKARI NEP-deep field
Aims. We aim to reveal environmental dependence of infrared luminosity functions (IR LFs) of galaxies at z ~ 0.8 using the AKARI
satellite. AKARI’s wide field of view and unique mid-IR filters help us to construct restframe 8 μm LFs directly without relying on
SED models.
Methods. We construct restframe 8 μm IR LFs in the cluster region RXJ1716.4+6708 at z = 0.81, and compare them with a blank
field using the AKARI north ecliptic pole deep field data at the same redshift. AKARI’s wide field of view (10' × 10') is suitable to
investigate wide range of galaxy environments. AKARI’s 15 μm filter is advantageous here since it directly probes restframe 8 μm at
z ~ 0.8, without relying on a large extrapolation based on a SED fit, which was the largest uncertainty in previous work.
Results. We have found that cluster IR LFs at restframe 8 μm have a factor of 2.4 smaller L^∗ and a steeper faint-end slope than that
of the field. Confirming this trend, we also found that faint-end slopes of the cluster LFs becomes flatter and flatter with decreasing
local galaxy density. These changes in LFs cannot be explained by a simple infall of field galaxy population into a cluster. Physics
that can preferentially suppress IR luminous galaxies in high density regions is required to explain the observed results
Surface Incompressibility from Semiclassical Relativistic Mean Field Calculations
By using the scaling method and the Thomas-Fermi and Extended Thomas-Fermi
approaches to Relativistic Mean Field Theory the surface contribution to the
leptodermous expansion of the finite nuclei incompressibility has been
self-consistently computed. The validity of the simplest expansion, which
contains volume, volume-symmetry, surface and Coulomb terms, is examined by
comparing it with self-consistent results of the finite nuclei
incompressibility for some currently used non-linear sigma-omega parameter
sets. A numerical estimate of higher-order contributions to the leptodermous
expansion, namely the curvature and surface-symmetry terms, is made.Comment: 18 pages, REVTeX, 3 eps figures, changed conten
Relative Expression Levels Rather Than Specific Activity Plays the Major Role in Determining In Vivo AKT Isoform Substrate Specificity
The AKT protooncogene mediates many cellular processes involved in normal development and disease states such as cancer. The three structurally similar isoforms: AKT1, AKT2, and AKT3 exhibit both functional redundancy and isoform-specific functions; however the basis for their differential signalling remains unclear. Here we show that in vitro, purified AKT3 is ∼47-fold more active than AKT1 at phosphorylating peptide and protein substrates. Despite these marked variations in specific activity between the individual isoforms, a comprehensive analysis of phosphorylation of validated AKT substrates indicated only subtle differences in signalling via individual isoforms in vivo. Therefore, we hypothesise, at least in this model system, that relative tissue/cellular abundance, rather than specific activity, plays the dominant role in determining AKT substrate specificity in situ
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