Low noise buffer amplifiers and buffered phase comparators for precise time and frequency measurement and distribution

Extremely low noise, high performance, wideband buffer amplifiers and buffered phase comparators were developed. These buffer amplifiers are designed to distribute reference frequencies from 30 KHz to 45 MHz from a hydrogen maser without degrading the hydrogen maser's performance. The buffered phase comparators are designed to intercompare the phase of state of the art hydrogen masers without adding any significant measurement system noise. These devices have a 27 femtosecond phase stability floor and are stable to better than one picosecond for long periods of time. Their temperature coefficient is less than one picosecond per degree C, and they have shown virtually no voltage coefficients

Multisystem proteinopathy due to a homozygous p.Arg159His VCP mutation : a tale of the unexpected

ObjectiveTo assess the clinical, radiologic, myopathologic, and proteomic findings in a patient manifesting a multisystem proteinopathy due to a homozygous valosin-containing protein gene (VCP) mutation previously reported to be pathogenic in the heterozygous state.MethodsWe studied a 36-year-old male index patient and his father, both presenting with progressive limb-girdle weakness. Muscle involvement was assessed by MRI and muscle biopsies. We performed whole-exome sequencing and Sanger sequencing for segregation analysis of the identified p.Arg159His VCP mutation. To dissect biological disease signatures, we applied state-of-the-art quantitative proteomics on muscle tissue of the index case, his father, 3 additional patients with VCP-related myopathy, and 3 control individuals.ResultsThe index patient, homozygous for the known p.Arg159His mutation in VCP, manifested a typical VCP-related myopathy phenotype, although with a markedly high creatine kinase value and a relatively early disease onset, and Paget disease of bone. The father exhibited a myopathy phenotype and discrete parkinsonism, and multiple deceased family members on the maternal side of the pedigree displayed a dementia, parkinsonism, or myopathy phenotype. Bioinformatic analysis of quantitative proteomic data revealed the degenerative nature of the disease, with evidence suggesting selective failure of muscle regeneration and stress granule dyshomeostasis.ConclusionWe report a patient showing a multisystem proteinopathy due to a homozygous VCP mutation. The patient manifests a severe phenotype, yet fundamental disease characteristics are preserved. Proteomic findings provide further insights into VCP-related pathomechanisms

Size-resolved aerosol emission factors and new particle formation/growth activity occurring in Mexico City during the MILAGRO 2006 Campaign

Measurements of the aerosol size distribution from 11 nm to 2.5 microns were made in Mexico City in March 2006, during the MILAGRO (Megacity Initiative: Local and Global Research Observations) field campaign. Observations at the urban supersite, referred to as T0, could often be characterized by morning conditions with high particle mass concentrations, low mixing heights, and highly correlated particle number and CO<sub>2</sub> concentrations, indicative that particle number is controlled by primary emissions. Average size-resolved and total number- and volume-based emission factors for combustion sources impacting T0 have been determined using a comparison of peak sizes in particle number and CO<sub>2</sub> concentration. Peaks are determined by subtracting the measured concentration from a calculated baseline concentration time series. The number emission and volume emission factors for particles from 11 nm to 494 nm are 1.56 × 10<sup>15</sup> particles, and 9.48 × 10<sup>11</sup> cubic microns per kg of carbon, respectively. The uncertainty of the number emission factor is approximately plus or minus 50 %. The mode of the number emission factor was between 25 and 32 nm, while the mode of the volume factor was between 0.25 and 0.32 microns. These emission factors are reported as log normal model parameters and are compared with multiple emission factors from the literature. In Mexico City in the afternoon, the CO<sub>2</sub> concentration drops during ventilation of the polluted layer, and the coupling between CO<sub>2</sub> and particle number breaks down, especially during new particle formation events when particle number is no longer controlled by primary emissions. Using measurements of particle number and CO<sub>2</sub> taken aboard the NASA DC-8, the determined primary emission factor was applied to the Mexico City Metropolitan Area (MCMA) plume to quantify the degree of secondary particle formation in the plume; the primary emission factor accounts for less than 50 % of the total particle number and the surplus particle count is not correlated with photochemical age. Primary particle volume and number in the size range 0.1–2 μm are similarly too low to explain the observed volume distribution. Contrary to the case for number, the apparent secondary volume increases with photochemical age. The size distribution of the apparent increase, with a mode at ~250 nm, is reported

Convective scaling of the average dissipation rate of temperature variance in the atmospheric surface layer

The flux of sensible heat from the land surface is related to the average rate of dissipation of temperature fluctuations in the atmospheric surface layer through the temperature variance budget equation. In many cases it is desirable to estimate the heat flux from measurement or inference of the dissipation rate. Here we study how the dissipation rate scales with atmospheric stability, using three inertial range methods to calculate the dissipation rate: power spectra, second order structure functions, and third order structure functions. Experimental data are analyzed from a pair of field experiments, during which turbulent fluctuations of velocity and temperature were measured over a broad range of neutral and unstable atmospheric flows. It is shown that the temperature dissipation rate scales with a single convective power law continuously from near-neutral to strongly unstable stratification. The dissipation scaling is found to nearly match production in the near-neutral region, but to be consistently lower than production in the more convective regimes. The convective scaling is shown to offer a simplified means of computing sensible heat flux from the dissipation rate of temperature variance

Vertical distribution of aerosols in the vicinity of Mexico City during MILAGRO-2006 Campaign

On 7 March 2006, a mobile, ground-based, vertical pointing, elastic lidar system made a North-South transect through the Mexico City basin. Column averaged, aerosol size distribution (ASD) measurements were made on the ground concurrently with the lidar measurements. The ASD ground measurements allowed calculation of the column averaged mass extinction efficiency (MEE) for the lidar system (1064 nm). The value of column averaged MEE was combined with spatially resolved lidar extinction coefficients to produce total aerosol mass concentration estimates with the resolution of the lidar (1.5 m vertical spatial and 1 s temporal). Airborne ASD measurements from DOE G-1 aircraft made later in the day on 7 March 2006, allowed the evaluation of the assumptions of constant ASD with height and time used for estimating the column averaged MEE. <br><br> The results showed that the aerosol loading within the basin is about twice what is observed outside of the basin. The total aerosol base concentrations observed in the basin are of the order of 200 μg/m<sup>3</sup> and the base levels outside are of the order of 100 μg/m<sup>3</sup>. The local heavy traffic events can introduce aerosol levels near the ground as high as 900 μg/m<sup>3</sup>. <br><br> The article presents the methodology for estimating aerosol mass concentration from mobile, ground-based lidar measurements in combination with aerosol size distribution measurements. An uncertainty analysis of the methodology is also presented

Tube Models for Rubber-Elastic Systems

In the first part of the paper we show that the constraining potentials introduced to mimic entanglement effects in Edwards' tube model and Flory's constrained junction model are diagonal in the generalized Rouse modes of the corresponding phantom network. As a consequence, both models can formally be solved exactly for arbitrary connectivity using the recently introduced constrained mode model. In the second part, we solve a double tube model for the confinement of long paths in polymer networks which is partially due to crosslinking and partially due to entanglements. Our model describes a non-trivial crossover between the Warner-Edwards and the Heinrich-Straube tube models. We present results for the macroscopic elastic properties as well as for the microscopic deformations including structure factors.Comment: 15 pages, 8 figures, Macromolecules in pres