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

Electromagnetic Moments of the Baryon Decuplet

We compute the leading contributions to the magnetic dipole and electric quadrupole moments of the baryon decuplet in chiral perturbation theory. The measured value for the magnetic moment of the $\Omega^-$ is used to determine the local counterterm for the magnetic moments. We compare the chiral perturbation theory predictions for the magnetic moments of the decuplet with those of the baryon octet and find reasonable agreement with the predictions of the large--$N_c$ limit of QCD. The leading contribution to the quadrupole moment of the $\Delta$ and other members of the decuplet comes from one--loop graphs. The pionic contribution is shown to be proportional to $I_z$ (and so will not contribute to the quadrupole moment of $I=0$ nuclei), while the contribution from kaons has both isovector and isoscalar components. The chiral logarithmic enhancement of both pion and kaon loops has a coefficient that vanishes in the $SU(6)$ limit. The third allowed moment, the magnetic octupole, is shown to be dominated by a local counterterm with corrections arising at two loops. We briefly mention the strange counterparts of these moments.Comment: Uses harvmac.tex, 15 pages with 3 PostScript figures packed using uufiles. UCSD/PTH 93-22, QUSTH-93-05, Duke-TH-93-5

MAR-Mediated transgene integration into permissive chromatin and increased expression by recombination pathway engineering.

Untargeted plasmid integration into mammalian cell genomes remains a poorly understood and inefficient process. The formation of plasmid concatemers and their genomic integration has been ascribed either to non-homologous end-joining (NHEJ) or homologous recombination (HR) DNA repair pathways. However, a direct involvement of these pathways has remained unclear. Here, we show that the silencing of many HR factors enhanced plasmid concatemer formation and stable expression of the gene of interest in Chinese hamster ovary (CHO) cells, while the inhibition of NHEJ had no effect. However, genomic integration was decreased by the silencing of specific HR components, such as Rad51, and DNA synthesis-dependent microhomology-mediated end-joining (SD-MMEJ) activities. Genome-wide analysis of the integration loci and junction sequences validated the prevalent use of the SD-MMEJ pathway for transgene integration close to cellular genes, an effect shared with matrix attachment region (MAR) DNA elements that stimulate plasmid integration and expression. Overall, we conclude that SD-MMEJ is the main mechanism driving the illegitimate genomic integration of foreign DNA in CHO cells, and we provide a recombination engineering approach that increases transgene integration and recombinant protein expression in these cells. Biotechnol. Bioeng. 2017;114: 384-396. © 2016 The Authors. Biotechnology and Bioengineering published by Wiley Periodicals, Inc

Magnetic Moments of Decuplet Baryons in Light Cone QCD

We calculate the magnetic moments of decuplet baryons containing strange quarks within the framework of light cone QCD sum rules taking into account the SU(3) flavor symmetry breaking effects. It is obtained that magnetic moments of the neutral \sso and \xis0 baryons are mainly determined by the SU(3) breaking terms. A comparison of our results on the magnetic moments of the decuplet baryons with the predictions of other approaches is presented.Comment: Latex, 20 pages, 6 figure

Understandings of sustainable corporate governance by Australian managed investment schemes and some implications for small-scale forestry in Australia

This study reveals that managers of Australian managed investment schemes understand sustainable corporate governance as a mix of financial, natural environment and social outcomes. The managers that were interviewed prioritized financial aspects of business performance but acknowledged that sustained financial performance was only possible if positive natural environment outcomes and positive social outcomes were also sustained. In this context, the managers expressed qualified support for the development of small-scale forestry in Australia

Magnetic moments of the SU(3) decuplet baryons in the chiral quark-soliton model

Magnetic moments of baryons are studied within the chiral quark soliton model with special emphasis on the decuplet of baryons. The model is used to identify all symmetry breaking terms proportional to $m_{\rm s}$. Sum rules for the magnetic moments are derived. A ``model-independent'' analysis of the symmetry breaking terms is performed and finally model calculations are presented, which show the importance of the rotational $1/N_{\rm c}$ corrections for cranking of the soliton.Comment: 22 pages, RevTex. The final version accepted for publication in Phys. Rev.

The Decuplet Revisited in χ\chiPT

The paper deals with two issues. First, we explore the quantitiative importance of higher multiplets for properties of the $\Delta$ decuplet in chiral perturbation theory. In particular, it is found that the lowest order one--loop contributions from the Roper octet to the decuplet masses and magnetic moments are substantial. The relevance of these results to the chiral expansion in general is discussed. The exact values of the magnetic moments depend upon delicate cancellations involving ill--determined coupling constants. Second, we present new relations between the magnetic moments of the $\Delta$ decuplet that are independent of all couplings. They are exact at the order of the chiral expansion used in this paper.Comment: 7 pages of double column revtex, no figure

Simple quark model with chiral phenomenology

We propose a new approach to the determination of hadronic observables in which the essential features of chiral symmetry are combined with conventional constituent quark models. To illustrate the approach, we consider the simple quark model in the limit of SU~3! flavor symmetry at the strange quark mass. The comparison with data is made after an analytic continuation which ensures the correct leading nonanalytic behavior of chiral perturbation theory. The approach not only gives an excellent fit for the octet baryon magnetic moments but the prediction for the D11 magnetic moment is also in good agreement with current measurements.I. C. Cloet, D. B. Leinweber, and A. W. Thoma