Serum Xylosyltransferase Activity in Diabetic Patients as a Possible Marker of Reduced Proteoglycan Biosynthesis

OBJECTIVE—Proteoglycan metabolism is altered in diabetic patients. The xylosyltransferases (XTs) are the initial and rate-limiting enzymes in the biosynthesis of the glycosaminoglycan chains in proteoglycans. Here, we analyzed whether the changed proteoglycan metabolism leads to altered serum XT levels in diabetic patients

The Energy Spectrum of TeV Gamma-Rays from the Crab Nebula as measured by the HEGRA system of imaging air Cherenkov telescopes

The Crab Nebula has been observed by the HEGRA (High-Energy Gamma-Ray Astronomy) stereoscopic system of imaging air Cherenkov telescopes (IACTs) for a total of about 200 hrs during two observational campaigns: from September 1997 to March 1998 and from August 1998 to April 1999. The recent detailed studies of system performance give an energy threshold and an energy resolution for gamma-rays of 500 GeV and ~ 18%, respectively. The Crab energy spectrum was measured with the HEGRA IACT system in a very broad energy range up to 20 TeV, using observations at zenith angles up to 65 degrees. The Crab data can be fitted in the energy range from 1 to 20 TeV by a simple power-law, which yields dJg/dE = (2.79+/-0.02 +/- 0.5) 10^{-7} E^{-2.59 +/- 0.03 +/- 0.05}, ph m^{-2} s^{-1} TeV^{-1} The Crab Nebula energy spectrum, as measured with the HEGRA IACT system, agrees within 15% in the absolute scale and within 0.1 units in the power law index with the latest measurements by the Whipple, CANGAROO and CAT groups, consistent within the statistical and systematic errors quoted by the experiments. The pure power-law spectrum of TeV gamma-rays from the Crab Nebula constrains the physics parameters of the nebula environment as well as the models of photon emission.Comment: to appear in ApJ, 29 pages, 6 figure

Correlated intense X-ray and TeV activity of Mrk~501 in 1998 June

We present exactly simultaneous X-ray and TeV monitoring with {\it RXTE} and HEGRA of the TeV blazar Mrk 501 during 15 days in 1998 June. After an initial period of very low flux at both wavelengths, the source underwent a remarkable flare in the TeV and X-ray energy bands, lasting for about six days and with a larger amplitude at TeV energies than in the X-ray band. At the peak of the TeV flare, rapid TeV flux variability on sub-hour timescales is found. Large spectral variations are observed at X-rays, with the 3--20 keV photon index of a pure power law continuum flattening from $\Gamma=2.3$ to $\Gamma=1.8$ on a timescale of 2--3 days. This implies that during the maximum of the TeV activity, the synchrotron peak shifted to energies $\gtrsim 50$ keV, a behavior similar to that observed during the longer-lasting, more intense flare in 1997 April. The TeV spectrum during the flare is described by a power law with photon index $\Gamma=1.9$ and an exponential cutoff at $\sim$ 4 TeV; an indication for spectral softening during the flare decay is observed in the TeV hardness ratios. Our results generally support a scenario where the TeV photons are emitted via inverse Compton scattering of ambient seed photons by the same electron population responsible for the synchrotron X-rays. The simultaneous spectral energy distributions (SEDs) can be fit with a one-zone synchrotron-self Compton model assuming a substantial increase of the magnetic field and the electron energy by a factor of 3 and 10, respectively.Comment: Accepted for publication in ApJ, Part

Simultaneous X-Ray and TeV Gamma-Ray Observations of the TeV Blazar Markarian 421 during February and May 2000

In this paper we present the results of simultaneous observations of the TeV blazar Markarian 421 (Mrk 421) at X-ray and TeV Gamma-ray energies with the Rossi X-Ray Timing Explorer (RXTE) and the stereoscopic Cherenkov Telescope system of the HEGRA (High Energy Gamma Ray Astronomy) experiment, respectively. The source was monitored from February 2nd to February 16th and from May 3rd to May 8th, 2000. We discuss in detail the temporal and spectral properties of the source. Remarkably, the TeV observations of February 7th/8th showed statistically significant evidence for substantial TeV flux variability on 30 min time scale. We show the results of modeling the data with a time dependent homogeneous Synchrotron Self-Compton (SSC) model. The X-ray and TeV gamma-ray emission strengths and energy spectra together with the rapid flux variability strongly suggest that the emission volume is approaching the observer with a Doppler factor of 50 or higher. The different flux variability time scales observed at X-rays and TeV Gamma-rays indicate that a more detailed analysis will require inhomogeneous models with several emission zones.Comment: Accepted for Publication in ApJ, 21 Pages, 5 Figure

The TeV Energy Spectrum of Mkn 501 Measured with the Stereoscopic Telescope System of HEGRA during 1998 and 1999

During 1997, the BL Lac object Mkn 501 went into an extraordinary state of high X-ray and TeV gamma-ray activity, lasting more than 6 months. In this paper we report on the TeV emission characteristics of the source in the subsequent years of 1998 and 1999 as measured with the Stereoscopic Cherenkov Telescope System of HEGRA (La Palma, Canary Islands). Our observations reveal a 1998-1999 mean emission level at 1 TeV of 1/3 of the flux of the Crab Nebula, a factor of 10 lower than during the year of 1997. A dataset of 122 observations hours with the HEGRA telescope system makes it possible to assess for the first time the Mkn 501 TeV energy spectrum for a mean flux level substantially below that of the Crab Nebula with reasonable statistical accuracy. Excluding the data of a strong flare, we find evidence that the 1998--1999 low-flux spectrum is substantially softer (by 0.44+-0.1(stat) in spectral index) than the 1997 time averaged spectrum. The 500 GeV to 10 TeV energy spectrum can well be described by a power law model with exponential cutoff: dN/dE ~ E^(-alpha) exp(-E/E0) with alpha=2.31+-0.22(stat), and E0=5.1 (-2.3+7.8)(stat) TeV. Within statistical accuracy, also a pure power law model gives an acceptable fit to the data: dN/dE ~ E^(-Gamma) with Gamma=2.76+-0.08(stat). After presenting the 1998-1999 TeV characteristics of the source we discuss the implications of the results.Comment: Accepted for publication in The Astrophysical Journal, Part 1, on August 4th, 200

Mutations in fam20b and xylt1 Reveal That Cartilage Matrix Controls Timing of Endochondral Ossification by Inhibiting Chondrocyte Maturation

Differentiating cells interact with their extracellular environment over time. Chondrocytes embed themselves in a proteoglycan (PG)-rich matrix, then undergo a developmental transition, termed “maturation,” when they express ihh to induce bone in the overlying tissue, the perichondrium. Here, we ask whether PGs regulate interactions between chondrocytes and perichondrium, using zebrafish mutants to reveal that cartilage PGs inhibit chondrocyte maturation, which ultimately dictates the timing of perichondral bone development. In a mutagenesis screen, we isolated a class of mutants with decreased cartilage matrix and increased perichondral bone. Positional cloning identified lesions in two genes, fam20b and xylosyltransferase1 (xylt1), both of which encode PG synthesis enzymes. Mutants failed to produce wild-type levels of chondroitin sulfate PGs, which are normally abundant in cartilage matrix, and initiated perichondral bone formation earlier than their wild-type siblings. Primary chondrocyte defects might induce the bone phenotype secondarily, because mutant chondrocytes precociously initiated maturation, showing increased and early expression of such markers as runx2b, collagen type 10a1, and ihh co-orthologs, and ihha mutation suppressed early perichondral bone in PG mutants. Ultrastructural analyses demonstrated aberrant matrix organization and also early cellular features of chondrocyte hypertrophy in mutants. Refining previous in vitro reports, which demonstrated that fam20b and xylt1 were involved in PG synthesis, our in vivo analyses reveal that these genes function in cartilage matrix production and ultimately regulate the timing of skeletal development

The Crab Nebula and Pulsar between 500 GeV and 80 TeV: Observations with the HEGRA stereoscopic air Cherenkov telescopes

The Crab supernova remnant has been observed regularly with the stereoscopic system of 5 imaging air Cherenkov telescopes that was part of the High Energy Gamma Ray Astronomy (HEGRA) experiment. In total, close to 400 hours of useful data have been collected from 1997 until 2002. The spectrum extends up to energies of 80 TeV and is well matched by model calculations in the framework of inverse Compton scattering of various seed photons in the nebula including for the first time a recently detected compact emission region at mm-wavelengths. The observed indications for a gradual steepening of the energy spectrum in data is expected in the inverse Compton emission model.The average magnetic field in the emitting volume is determined to be $(161.6\pm0.8mathrm{stat}\pm18_\mathrm{sys}) \mu$G. The presence of protons in the nebula is not required to explain the observed flux and upper limits on the injected power of protons are calculated being as low as 20 % of the total spin down luminosity for bulk Lorentz factors of the wind in the range of $10^4-10^6$.The position and size of the emission region have been studied over a wide range of energies. The position is shifted by 13\arcsec to the west of the pulsar with a systematic uncertainty of 25\arcsec. No significant shift in the position with energy is observed. The size of the emission region is constrained to be less than 2\arcmin at energies between 1 and 10 TeV. Above 30 TeV the size is constrained to be less than 3\arcmin.No indications for pulsed emission has been found and upper limits in differential bins of energy have been calculated reaching typically 1-3 % of the unpulsed component.Comment: 53 pages, 12 figures. Accepted for publication in Astrophysical Journa