2,974 research outputs found
Stability of the Submillimeter Brightness of the Atmosphere Above Mauna Kea, Chajnantor and the South Pole
The summit of Mauna Kea in Hawaii, the area near Cerro Chajnantor in Chile,
and the South Pole are sites of large millimeter or submillimeter wavelength
telescopes. We have placed 860 GHz sky brightness monitors at all three sites
and present a comparative study of the measured submillimeter brightness due to
atmospheric thermal emission. We report the stability of that quantity at each
site.Comment: 6 figure
Methionine Adenosyltransferase I/III Deficiency in Portugal: High Frequency of a Dominantly Inherited Form in a Small Area of Douro High Lands
Methionine adenosyltransferase deficienc(MAT I/III deficiency) is an inborn error of metabolism resulting in isolated hypermethioninemia, and usually inherited as an autosomal recessive trait, although a dominant form has been reported in several families. During the last 6 years, approximately 520,000 newborns were screened in the Portuguese Newborn Screening Laboratory by MS/MS, and 21 cases of persistent hypermethioninemia were found. One case was confirmed to be a deficiency of cystathionine b-synthase and 20 cases were confirmed by MAT1A gene analysis to have an elevation of methionine due to MAT I/III deficiency, which indicates an incidence for this condition of 1/26,000. Twelve of the MAT I/III deficient newborns, belonging to 11 families, were identified in the northern region of Portugal and sent to the same treatment center, where they are under follow-up. Clinical, biochemical, and genetic characteristics of individuals from these 11 families are presented. Plasma methionine and homocysteine concentrations were found to be moderately increased in all
newborns, and molecular analysis revealed that they all were heterozygous for R264H mutation. Normal growth,development, and neurological examination were observed in all cases, and cerebral MRI performed in six cases revealed myelination abnormalities in one case. Plasma methionine concentration for all 12 cases was always below 300 mM, and they are all on a normal diet for their age
The wintertime South Pole tropospheric water vapor column: Comparisons of radiosonde and recent terahertz radiometry, use of the saturated column as a proxy measurement, and inference of decadal trends
We use a fifty-year record of wintertime radiosonde observations at the South Pole to estimate the precipitable water vapor column (PWV) over the entire period. Humidity data from older radiosondes is of limited reliability; however, we think an estimation of PWV is possible using temperature data because the wintertime lower troposphere is very close to saturated. From temperature data we derived PWV_SAT which is the PWV if the troposphere was saturated over the entire column. Comparisons to recent radiosonde humidity data indicate that PWV ≃ 0.88PWV_SAT. Since 1998 a CMU/NRAO 860 GHz atmospheric radiometer has been operating at the South Pole producing zenith opacity data, τo. It is expected that τo ∝ PWV, and also τ_o ∝ PWV_SAT, since the lower atmospheric column is near to saturation. We compare trends in τo, PWV_SAT, and PWV. PWV and PWV_SAT showed little trend in the last fifty years, 1961 to 2010, except perhaps in the last two decades, when PWVSAT was below average, followed by an increasing trend to above average. This increasing trend in the last decade was also observed in τo, except for the final two years when it appears that something changed in the instrument response. PWV_SAT is a useful metric for estimating PWV in the earlier years of wintertime South Pole radiosonde, and it is generally useful for evaluating the wintertime performance of radiosonde humidity and atmospheric opacity instrumentation
In Solidarity
This edition of Next Page is a departure from our usual question and answer format with a featured campus reader. Instead, we asked speakers who participated in the College’s recent Student Solidarity Rally (March 1, 2017) to recommend readings that might further our understanding of the topics on which they spoke
Timing molecular motion and production with a synthetic transcriptional clock
The realization of artificial biochemical reaction networks with unique functionality is one of the main challenges for the development of synthetic biology. Due to the reduced number of components, biochemical circuits constructed in vitro promise to be more amenable to systematic design and quantitative assessment than circuits embedded within living organisms. To make good on that promise, effective methods for composing subsystems into larger systems are needed. Here we used an artificial biochemical oscillator based on in vitro transcription and RNA degradation reactions to drive a variety of “load” processes such as the operation of a DNA-based nanomechanical device (“DNA tweezers”) or the production of a functional RNA molecule (an aptamer for malachite green). We implemented several mechanisms for coupling the load processes to the oscillator circuit and compared them based on how much the load affected the frequency and amplitude of the core oscillator, and how much of the load was effectively driven. Based on heuristic insights and computational modeling, an “insulator circuit” was developed, which strongly reduced the detrimental influence of the load on the oscillator circuit. Understanding how to design effective insulation between biochemical subsystems will be critical for the synthesis of larger and more complex systems
Universal Power Law in the Noise from a Crumpled Elastic Sheet
Using high-resolution digital recordings, we study the crackling sound
emitted from crumpled sheets of mylar as they are strained. These sheets
possess many of the qualitative features of traditional disordered systems
including frustration and discrete memory. The sound can be resolved into
discrete clicks, emitted during rapid changes in the rough conformation of the
sheet. Observed click energies range over six orders of magnitude. The measured
energy autocorrelation function for the sound is consistent with a stretched
exponential C(t) ~ exp(-(t/T)^{b}) with b = .35. The probability distribution
of click energies has a power law regime p(E) ~ E^{-a} where a = 1. We find the
same power law for a variety of sheet sizes and materials, suggesting that this
p(E) is universal.Comment: 5 pages (revtex), 10 uuencoded postscript figures appended, html
version at http://rainbow.uchicago.edu/~krame
Extreme Ultraviolet Variability Experiment (EVE) Multiple EUV Grating Spectrographs (MEGS): Radiometric Calibrations and Results
The NASA Solar Dynamics Observatory (SDO), scheduled for launch in early 2010, incorporates a suite of instruments including the Extreme Ultraviolet Variability Experiment (EVE). EVE has multiple instruments including the Multiple Extreme ultraviolet Grating Spectrographs (MEGS) A, B, and P instruments, the Solar Aspect Monitor (SAM), and the Extreme ultraviolet SpectroPhotometer (ESP). The radiometric calibration of EVE, necessary to convert the instrument counts to physical units, was performed at the National Institute of Standards and Technology (NIST) Synchrotron Ultraviolet Radiation Facility (SURF III) located in Gaithersburg, Maryland. This paper presents the results and derived accuracy of this radiometric calibration for the MEGS A, B, P, and SAM instruments, while the calibration of the ESP instrument is addressed by Didkovsky et al. . In addition, solar measurements that were taken on 14 April 2008, during the NASA 36.240 sounding-rocket flight, are shown for the prototype EVE instruments
Caenorhabditis briggsae recombinant inbred line genotypes reveal inter-strain incompatibility and the rvolution of recombination
Newborn Screening for Homocystinuria Revealed a High Frequency of MAT I/III Deficiency in Iberian Peninsula
Acessível em: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4375120/Homocystinuria due to cystathionine β-synthase deficiency or "classical homocystinuria" is a rare autosomal recessive condition resulting in altered sulfur metabolism with elevated methionine and homocysteine in plasma and homocystine in urine. This condition is characterized by a high clinical heterogeneity, which contributes to late clinical diagnosis, usually only made after irreversible damage has occurred. Treatment is effective if started before clinical symptoms. The analysis of methionine levels by tandem mass spectrometry (MS/MS) allows the newborn screening for homocystinuria, but false-positive results can be frequently obtained and lead to the unwanted identification of methionine adenosyl transferase (MAT I/III) deficiency. This latter condition is biochemically characterized by isolated persistent hypermethioninemia, accompanied in some individuals with slightly elevated levels of homocysteine in plasma. A dominant form of MAT I/III deficiency, associated with mutation p.R264H, seems to be very frequent in the Iberian Peninsula and usually has a clinically benign course. Both these metabolic disorders are screened in Galicia and Portugal since the introduction of the MS/MS technology, in 2000 and 2004, respectively, resulting in the identification of three patients with classical homocystinuria and 44 patients with MAT I/III deficiency. All but one heterozygous parent of MAT I/III patients, identified with the p.R264H mutation, are healthy adults around the age of 30/40. The implementation of a second-tier test for homocysteine in dried blood spots would considerably reduce the number of MAT I/III-deficient patients identified and improve the specificity and positive predictive value for classical homocystinuria screening
- …
