Elevated serum biotinidase activity in hepatic glycogen storage disorders-A convenient biomarker

Summary: An elevated serum biotinidase activity in patients with glycogen storage disease (GSD) type Ia has been reported previously. The aim of this work was to investigate the specificity of the phenomenon and thus we expanded the study to other types of hepatic GSDs. Serum biotinidase activity was measured in a total of 68 GSD patients and was compared with that of healthy controls (8.7 ±10; range 7.0-10.6mU/ml; n=6). We found an increased biotinidase activity in patients with GSD Ia (17.7 ±3.9; range: 11.4-24.8; n=21), GSD I non-a (20.9 ±5.6; range 14.6-26.0; n=4), GSD III (12.5 ±-3.6; range 7.8-19.1; n=3), GSD VI (15.4 ±-2.0; range 14.1-17.7; n=) and GSD IX (14.0 ±-3.8; range: 7.5-21.6; n=22). The sensitivity of this test was 100% for patients with GSD Ia, GSD I non-a and GSD VI, 62% for GSD III, and 77% for GSD IX, indicating reduced sensitivity for GSD III and GSD IX, respectively. In addition, we found elevated biotinidase activity in all sera from 5 patients with Fanconi-Bickel Syndrome (15.3 ±-3.7; range 11.0-19.4). Taken together, we propose serum biotinidase as a diagnostic biomarker for hepatic glycogen storage disorder

Cryptococcus gattii Meningoencephalitis in an Immunocompetent Person 13 Months after Exposure

Abstract : A 53-year old immunocompetent Swiss female is described who developed severe meningoencephalitis due to infection with Cryptococcus gattii 13 months following exposure on Vancouver Island, Canada. Diagnosis was based on cerebrospinal fluid (CSF) examination, i.e., positive India-ink staining, positive latex particle agglutination, and positive culture. Species identification was performed by growth on L-canavanine-glycine-bromthymol blue medium and by sequencing of the intergenic and internal transcribed spacer regions of the rRNA genes. After initial therapy with fluconazole by which the patient did not improve, therapy was changed to amphotericin B and flucytosine and later to high-dose fluconazole and amphotericin B. Despite long-term treatment and external drainage of the CSF, the patient's condition improved only slowly. The patient was discharged after 132 days of hospitalizatio

Magnetic Moments of the Baryon Decuplet in a Relativistic Quark Model

The magnetic moments of the baryon decuplet are calculated in a relativistic constituent quark model using the light-front formalism. Of particular interest are the magnetic moments of the $\Omega^-$ and $\Delta^{++}$ for which new recent experimental measurements are available. Our calculation for the magnetic moment ratio $\mu(\Delta^{++})/\mu(p)$ is in excellent agreement with the experimental ratio, while our ratio $\mu(\Omega^-)/\mu(\Lambda^0)$ is slightly higher than the experimental ratio.Comment: 10 pages ReVTeX, SLAC-PUB-621

Characterization of fecal nitrogen forms produced by a sheep fed with 15N labeled ryegrass

Little is known about nitrogen (N) forms in ruminant feces, although this information is important to understand N dynamics in agro-ecosystems. We fed 15N labeled ryegrass hay to a sheep and collected 15N labeled feces. Nitrogen forms in the feces were characterized by chemical extractions, solid-state cross polarization 15N nuclear magnetic resonance spectroscopy (SS CP/MAS 15N NMR) and Curie-point pyrolysis-gas chromatography/mass spectrometry (Cp Py-GC/MS). A 4months incubation experiment was conducted to assess N release from the feces. Half of the fecal N could be ascribed to bacterial and endogenous debris and a third to undigested dietary N. About a tenth of the fecal N was mineralized during the incubation experiment. The 15N abundance of nitrate released during the incubation remained constant and close to the 15N abundance of the total feces N. The NMR analysis of the feces showed that most of the N was present in proteins, while some was present as heterocyclic N, amino acids and ammonium. The Cp Py-GC/MS analysis confirmed the presence of proteins, amino acids and heterocyclic N in the feces. Comparing these results to those obtained from the 15N labeled hay suggests that some N compounds present in the plant were not digested by the animal, and that the animal excreted de novo synthesized N compounds. The low content in ammonium and amino acids, the low rate of N release from these feces during the incubation and the relatively high fecal protein content, particularly the hard to mineralize undigested and microbially bound forms, can explain the low transfer of N from these feces to crops observed in a previous wor

Value of FDG-PET/MR in Oral Focus Assessment in Head and Neck Cancer Patients-A Feasibility Study

FDG-PET/MR is a hybrid imaging modality used for the staging and restaging of advanced head & neck cancer (HNC) patients. Their treatment typically involves radiation therapy, which requires previous dental focus assessment. The aim of this study was to analyze if staging FDG-PET/MR is a valuable tool for oral focus assessment. For this purpose, FDG-PET/MR findings, such as metabolic activity of periapical radiolucencies and marginal periodontitis, were retrospectively compared with conventional standardized dental focus assessment, including dental radiographs and clinical assessment of 124 teeth in seven patients. Increased FDG uptake of periapical lesions was found in one out of 23 lesions. Increased FDG uptake of the marginal periodontium was recorded in one out of 34 lesions. In summary, standardized dental focus assessment by panoramic radiography and periapical radiographs may be enriched by information from FDG-PET/MR, showing active inflammation in dental foci. However, many dental foci have no correlate in FDG-PET/MR. The treatment decision for oral foci may benefit from the visualized presence or absence of metabolic activity on FDG-PET/MR

Trophic factor BDNF inhibits GABAergic signaling by facilitating dendritic enrichment of SUMO E3 ligase PIAS3 and altering gephyrin scaffold

Posttranslational addition of a small ubiquitin-like modifier (SUMO) moiety (SUMOylation) has been implicated in pathologies such as brain ischemia, diabetic peripheral neuropathy, and neurodegeneration. However, nuclear enrichment of SUMO pathway proteins has made it difficult to ascertain how ion channels, proteins that are typically localized to and function at the plasma membrane, and mitochondria are SUMOylated. Here, we report that the trophic factor, brain-derived neurotrophic factor (BDNF) regulates SUMO proteins both spatially and temporally in neurons. We show that BDNF signaling via the receptor tropomyosin-related kinase B facilitates nuclear exodus of SUMO proteins and subsequent enrichment within dendrites. Of the various SUMO E3 ligases, we found that PIAS-3 dendrite enrichment in response to BDNF signaling specifically modulates subsequent ERK1/2 kinase pathway signaling. In addition, we found the PIAS-3 RING and Ser/Thr domains, albeit in opposing manners, functionally inhibit GABA-mediated inhibition. Finally, using oxygen-glucose deprivation as an in vitro model for ischemia, we show that BDNF-tropomyosin-related kinase B signaling negatively impairs clustering of the main scaffolding protein at GABAergic postsynapse, gephyrin, whereby reducing GABAergic neurotransmission postischemia. SUMOylation-defective gephyrin K148R/K724R mutant transgene expression reversed these ischemia-induced changes in gephyrin cluster density. Taken together, these data suggest that BDNF signaling facilitates the temporal relocation of nuclear-enriched SUMO proteins to dendrites to influence postsynaptic protein SUMOylation

Characterisation of the mycobacterial NER system reveals novel functions of uvrD1 helicase

In this study, we investigated the role of the nucleotide excision repair (NER) pathway in mycobacterial DNA repair. Mycobacterium smegmatis lacking the NER excinuclease component uvrB, the helicase uvrD1 and a double knock-out lacking both proteins were constructed and their sensitivity to a series of DNA damaging agents wa analysed. As anticipated, the mycobacterial NER system was shown to be involved in the processing of bulky DNA adducts and inter-strand cross-links. In addition, it could be shown to exert a protective effect against oxidising and nitrosating agents. Interestingly, inactivation of uvrB and uvrD1 significantly increased marker integration frequencies in gene conversion assays. This implies that in mycobacteria, which lack the postreplicative mismatch repair system, NER, and particularly the UvrD1 helicase, is involved in the processing of a subset of recombination-associated mismatches

Unique properties of quadratic solitons

Quadratic spatial solitons exist in media with second order nonlinearities near the phase-matching condition for frequency mixing processes involving two or three waves of different frequency. Discussed here are a number of properties of these special solitons which are different from those of other spatial solitons which rely on optically induced index changes for guiding. First, the self-guiding properties of quadratic solitons are shown to have completely different origins than solitons which rely on index changes. Second, it is shown that there exists a large variety of quadratic solitons which contain two or three distinct spectral components with relative amplitudes depending on the phase mismatch, dimensionality of the propagation geometry, the soliton power and the launching conditions. Third, under appropriate conditions, solitons can be formed even when the group velocity directions for the spectral components lead to walk-off under normal circumstances. Fourth, for type II phase-matching in bulk crystals, seeded interactions lead to saturating amplifier characteristics

Quark Orbital Angular Momentum in the Baryon

Analytical and numerical results, for the orbital and spin content carried by different quark flavors in the baryons, are given in the chiral quark model with symmetry breaking. The reduction of the quark spin, due to the spin dilution in the chiral splitting processes, is transferred into the orbital motion of quarks and antiquarks. The orbital angular momentum for each quark flavor in the proton as a function of the partition factor $\kappa$ and the chiral splitting probability $a$ is shown. The cancellation between the spin and orbital contributions in the spin sum rule and in the baryon magnetic moments is discussed.Comment: 26 pages, 3 figures, revised version with minor eq. no and ref. no. corrections. Discussion on the $\Lambda$ spin and a new ref. are adde

The reaction γp→π∘γ′p\gamma p \to \pi^\circ \gamma^\prime p and the magnetic dipole moment of the Δ+(1232)\Delta^+(1232) resonance

The reaction $\gamma p \to \pi^\circ \gamma^\prime p$ has been measured with the TAPS calorimeter at the Mainz Microtron accelerator facility MAMI for energies between $\sqrt{s}$ = 1221--1331 MeV. Cross sections differential in angle and energy have been determined for all particles in the final state in three bins of the excitation energy. This reaction channel provides access to the magnetic dipole moment of the $\Delta^{+}(1232)$ resonance and, for the first time, a value of $\mu_{\Delta^+} = (2.7_{-1.3}^{+1.0}(stat.) \pm 1.5 (syst.) \pm 3(theo.)) \mu_N$ has been extracted