Serum hexosaminidase and ß-glucuronidase activities in infants: effects of age and sex

2003

We investigated the effect of age and sex on the serum activity of hexosaminidase (HEX) and ß-glucuronidase (BGLU) in 275 normal term infants aged 12 h to 12 months. Up to six weeks of life, HEX was significantly higher in boys (P<=0.023). During the age period of 1-26 weeks, BGLU was also higher in boys, but differences were significant only at 2-6 and 7-15 weeks (P<=0.016). The developmental pattern of HEX and BGLU was sex dependent. HEX activity increased in both sexes from 4-7 days of life, reaching a maximum of 1.4-fold the birth value at 2-6 weeks of age in boys (P<0.001) and a maximum of 1.6-fold at 7-15 weeks in girls (P<0.001). HEX activity gradually decreased thereafter, reaching …

Mortality and cause of death in mucopolysaccharidosis type II-a historical review based on data from the Hunter Outcome Survey (HOS).

2009

Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a progressive, multisystemic disease caused by a deficiency of iduronate-2-sulfatase. Patients with the severe form of the disease have cognitive impairment and typically die in the second decade of life. Patients with the less severe form do not experience significant cognitive involvement and may survive until the fifth or sixth decade of life. We studied the relationship of both severity of MPS II and the time period in which patients died with age at death in 129 patients for whom data were entered retrospectively into HOS (Hunter Outcome Survey), the only large-scale, multinational observational study of patients with MPS II.…

Calibration of high voltages at the ppm level by the difference of $$^{83{\mathrm{m}}}$$ 83m Kr conversion electron lines at the KATRIN experiment

2018

The neutrino mass experiment KATRIN requires a stability of 3 ppm for the retarding potential at − 18.6 kV of the main spectrometer. To monitor the stability, two custom-made ultra-precise high-voltage dividers were developed and built in cooperation with the German national metrology institute Physikalisch-Technische Bundesanstalt (PTB). Until now, regular absolute calibration of the voltage dividers required bringing the equipment to the specialised metrology laboratory. Here we present a new method based on measuring the energy difference of two $$^{83{\mathrm{m}}}$$ 83m Kr conversion electron lines with the KATRIN setup, which was demonstrated during KATRIN’s commissioning measurements …

Melting the Superconducting State in the Electron Doped Cuprate Pr$_{1.85}% $Ce$_{0.15}$CuO$_{4-\delta}$ with Intense near-infrared and Terahertz Pul…

2016

We studied the superconducting (SC) state depletion process in an electron doped cuprate Pr$_{1.85}$Ce$_{0.15}$CuO$_{4-\delta}$ by pumping with near-infrared (NIR) and narrow-band THz pulses. When pumping with THz pulses tuned just above the SC gap, we find the absorbed energy density required to deplete superconductivity, $A_{dep}$, matches the thermodynamic condensation energy. Contrary, by NIR pumping $A_{dep}$ is an order of magnitude higher, despite the fact that the SC gap is much smaller than the energy of relevant bosonic excitations. The result implies that only a small subset of bosons contribute to pairing.

The summertime Boreal forest field measurement intensive (HUMPPA-COPEC-2010): an overview of meteorological and chemical influences

2011

This paper describes the background, instrumentation, goals, and the regional influences on the HUMPPACOPEC intensive field measurement campaign, conducted at the Boreal forest research station SMEAR II (Station for Measuring Ecosystem-Atmosphere Relation) in Hyyti¨al¨a, Finland from 12 July–12 August 2010. The prevailing meteorological conditions during the campaign are examined and contrasted with those of the past six years. Back trajectory analyses show that meteorological conditions at the site in 2010 were characterized by a higher proportion of southerly flow than in the other years studied. As a result the summer of 2010 was anomalously warm and high in ozone making the campaign rel…

In-vitro Proteoglykansynthese in redifferenzierten Chondrozyten

1989

Human chondrocytes growing in monolayer cultures de-differentiate and produce type I collagen. They re-differentiate and resume their in-vivo characteristics (including the production of type II collagen) when cultured in an agarose-gel. To characterize the modulated cells in more detail, biochemical studies were performed in chondrocytes suspended in agarose for 1 to 3 weeks.

Efficacy of NaOCl/H2O2irrigation and GaAlAs laser in decontamination of root canals in vitro

2003

Background and Objectives To investigate the bactericidal effect of an 809 nm semiconductor laser alone, and in combination with NaOCl/H2O2 irrigation in root canals in vitro. Study Design/Materials and Methods A total of 72 human single-rooted teeth extracted for periodontal reasons were included. The crowns were removed, the roots shortened to a length of 12 mm, and the canals enlarged up to an apical size of #50 file. The specimens were autoclaved and incubated with a suspension of Streptococcus sanguinis (ATCC 10556). Laser irradiation was performed on a PC-controlled XY translation stage. A 200 micron optic fiber was used. Twelve specimens were irradiated at a power output of 1.5, 3.0,…

SARS-CoV-2 vaccination modelling for safe surgery to save lives: data from an international prospective cohort study

2021

Abstract Background Preoperative SARS-CoV-2 vaccination could support safer elective surgery. Vaccine numbers are limited so this study aimed to inform their prioritization by modelling. Methods The primary outcome was the number needed to vaccinate (NNV) to prevent one COVID-19-related death in 1 year. NNVs were based on postoperative SARS-CoV-2 rates and mortality in an international cohort study (surgical patients), and community SARS-CoV-2 incidence and case fatality data (general population). NNV estimates were stratified by age (18–49, 50–69, 70 or more years) and type of surgery. Best- and worst-case scenarios were used to describe uncertainty. Results NNVs were more favourable in su…

Improved Upper Limit on the Neutrino Mass from a Direct Kinematic Method by KATRIN

2019

We report on the neutrino mass measurement result from the first four-week science run of the Karlsruhe Tritium Neutrino experiment KATRIN in spring 2019. Beta-decay electrons from a high-purity gaseous molecular tritium source are energy analyzed by a high-resolution MAC-E filter. A fit of the integrated electron spectrum over a narrow interval around the kinematic end point at 18.57 keV gives an effective neutrino mass square value of (−1.0−1.1+0.9) eV2. From this, we derive an upper limit of 1.1 eV (90% confidence level) on the absolute mass scale of neutrinos. This value coincides with the KATRIN sensitivity. It improves upon previous mass limits from kinematic measurements by almost a …

Der Einfluß von N-Azetylglucosamin auf die Proteoglykansynthese normaler und arthrotischer Chondrozyten

1991

Die Arthrose geht mit einem Verlust der biologischen Eigenschaften des Gelenk-Knorpels einher. Die Bildung der Hauptkomponenten der extrazellularen Matrix (Kollagen und Proteoglykane) ist beeintrachtigt. Zur Analyse der metabolischen Veranderungen im Verlaufe verschiedener Arthrosestadien wurden biochemische Analysen an kultivierten Chondrozyten durchgefuhrt Da Chondrozyten im Verlaufe der Subkultivierung entdifferenzieren, war es erforderlich, sie zur Redifferenzierung in eine Agarosematrix zu uberfuhren. Aus fruheren Untersuchungen ist bekannt, das durch diese Technik der ursprungliche Phanotyp der Knorpelzelle wiederhergestellt werden kann.

Gamma-induced background in the KATRIN main spectrometer

2019

The KATRIN experiment aims to measure the effective electron antineutrino mass $$m_{\overline{\nu }_e}$$ mν¯e with a sensitivity of $${0.2}\,{\hbox {eV}/\hbox {c}^2}$$ 0.2eV/c2 using a gaseous tritium source combined with the MAC-E filter technique. A low background rate is crucial to achieving the proposed sensitivity, and dedicated measurements have been performed to study possible sources of background electrons. In this work, we test the hypothesis that gamma radiation from external radioactive sources significantly increases the rate of background events created in the main spectrometer (MS) and observed in the focal-plane detector. Using detailed simulations of the gamma flux in the e…

Gaucher's disease: therapy by intravenous infusions of modified glucocerebrosidase

1993

Deciphering the conformations and dynamics of FG-nucleoporins in situ

2022

AbstractThe ∼120 MDa nuclear pore complex (NPC) acts as a gatekeeper for the molecular traffic between the nucleus and the cytosol. Small cargo readily passes through the transport channel, yet large cargo requires specialized nuclear transport receptors. While the scaffold structure that anchors the NPC in the double-layered nuclear envelope has been resolved to remarkable details, the spatial organization of intrinsically disordered nucleoporins (NUPs) within the central channel remains enigmatic. These so-called FG-NUPs account for about one-third of the total mass of the NPC and form the actual transport barrier. Here we combined site-specific fluorescent labeling in non-fixed cells and…

Preparation methods to optimize the performance of sensor discs for fast chemiluminescence ozone analyzers.

2013

Fast ozone (O(3)) measurements (1-50 Hz) in the atmosphere are required for airborne studies and for the measurement of ground-based O(3) fluxes by the eddy covariance technique. Fast response analyzers, based on heterogeneous chemiluminescence, need dye coated sensor discs on which the chemiluminescence is generated. In this study, we present three new preparation methods for those sensor discs. Currently available sensor discs exhibit a fast temporal decay of sensitivity, resulting in short duty times which is troublesome for many field applications. To produce sensor discs that provide more stable signals over time, three dyes and nine energy transfer reagents were tested (as well as dif…

"Table 3" of "Spectra and correlations of Lambda and Lambda produced in 340-GeV/c Sigma -+C and 260-GeV/c n+C interactions"

2002

Total inclusive LAMBDA LAMBDA and LAMBDABAR LAMBDABAR pair production crosssections for the SIGMA- beam on the Carbon target.

"Table 1" of "Spectra and correlations of Lambda and Lambda produced in 340-GeV/c Sigma -+C and 260-GeV/c n+C interactions"

2002

Total inclusive LAMBDA and LAMBDABAR production cross sections for the SIGMA- beam on the Carbon target.

"Table 4" of "Spectra and correlations of Lambda and Lambda produced in 340-GeV/c Sigma -+C and 260-GeV/c n+C interactions"

2002

Total inclusive LAMBDA LAMBDA pair production cross sections for the Neutron beam on the Carbon target.

"Table 2" of "Spectra and correlations of Lambda and Lambda produced in 340-GeV/c Sigma -+C and 260-GeV/c n+C interactions"

2002

Total inclusive LAMBDA and LAMBDABAR production cross sections for the Neutron beam on the Carbon target.