Patients with Fabry disease (FD) are at a high risk for developing chronic kidney disease and cardiovascular disease. The availability of specific but costly therapy has elevated the profile of this rare condition. This KDIGO conference addressed controversial areas in the diagnosis, screening, and management of FD, and included enzyme replacement therapy and nonspecific standard-of-care therapy for the various manifestations of FD. Despite marked advances in patient care and improved overall outlook, there is a need to better understand the pathogenesis of this glycosphingolipidosis and to determine the appropriate age to initiate therapy in all types of patients. The need to develop more effective specific therapies was also emphasized.
Lipases from Candida antarctica and Mucor miehei were encapsulated in lecithin water-in-oil (w/o) microemulsion-based organogels (MBG). These gels were formulated with either hydroxypropylmethyl cellulose (HPMC) or gelatin. The esterification of lauric acid and 1-propanol catalyzed by these MBGs was examined in supercritical carbon dioxide (scCO 2 ; 35• C, 110 bar) as solvent for the substrates. The results were compared to those obtained with the reference substrate solvent isooctane. It turned out that the initial rates of this model reaction in scCO 2 were higher than those observed in the reference system. Various parameters affecting the biocatalysis such as pressure, alcohol and acid chain length, and gel composition were investigated. Kinetic studies showed that the ester synthesis catalyzed by the immobilized C. antarctica lipase occurs via a Ping Pong Bi Bi mechanism in which only inhibition by excess of alcohol was identified. Values of all kinetic parameters were determined. In addition, experiments on the reusability of these gels in scCO 2 were carried out and the state of water within the organogel was examined with the help of differential scanning calorimetry. The present study shows that biocatalysis using MBGs in scCO 2 is a promising alternative to other bioconversion processes.
Dedicated to Professor Gerhard Fritz on the occasion Of his 60th birthday The dicyanophosphide ion P(CN)y is formed on reduction of phosphorus tricyanide, e.g. with [18]crown-6-sodium cation surrounded by a crown ether and situated at a crystallographic inversion center (0.5, 0, 0.5 and 0, 0.5, 0.5, respectively), which takes up two further ligands in the trans position, namely two 0-coordinated tetrahydrofuran molecules or two N-coordinated dicyanophosphide ions (Fig. l The calculated net densities show that the anionic charge is distributed completely over the two cyanide residues while the phosphorus remains free. The high population on N corresponds to the (charge-controlled) N-coordination of P(CN) y to Na+ in the structure described 934
13C, 14N and 29Si-n.m.r. data of azo compounds R1-N=N-R2 with substituents Ri = R2 = N(CH3)2, NC5Hlo, N[Si(CH3)3]2, OSi(CH3)3, Si(CH3)3 and Rl/Rz = CH3/Si(CH3)3, C(CH3)3/Si(CH3)3, C(CH3)3/Si(OCH3)3, C(CH3I3 /Ge(CH3)3, C(CH3I3 /P(CH3)2 and C ~H S/ Si(CH3)3 are reported and compared with correspondmg values of other compounds known from the literature. The chemical shifts 614N show an almost linear correlation with n -+ n* transition energies while no dependence on calculated charge densities is observed. This demonstrates that the low energy transitions n + w* are mainly responsible for nitrogen shielding of azo compounds. 614N of bis(trimethylsi1yl)di-imine appears to be at the lowest field of all diamagnetic nitrogen compounds so far studied. The orbital sequence in azo compounds is checked by means of photoelectron (pee.) spectroscopy. The hitherto unknown p.e. spectra of some azo compounds are discussed qualitatively and by means of CNDO/S calculations. It is shown that n.m.r., U.V. and p.e. spectroscopy are sensitive to substituent effects in azo compounds.
1909* In this paper, bold figures refer to the compounds listed in table 1.
Von 16 Benzol-, Naphthalin-und Styrol-Derivaten mit Substituenten X = SCH,, CH2SCH3, Photoelectron Spectra and Molecular Properties, XN'*2) The Sulfur Lone Pair Delocalization in CHJS-Substituted Aromatic Systems Photoelectron (PE) spectra and charge transfer excitation energies of 16 derivatives of benzene, naphthalene and styrene with substituents X = SCH3, CHzSCH3, OCH3, and CH20CH3 are discussed. PE-splitting patterns can be understood qualitatively as x-perturbations, and can be explained quantitatively in terms of x-SCF-calculations. The PE ionization potentials of sulfides can be linearly correlated with the CT excitation energies of their Q-and x-complexes with tetracyanoethylene. In summary, the delocalisation of the sulfur lone pair in the ground state of aromatic x-systems can be described in terms of a ,,p-model" neglecting ,,p,Jdx" back-bonding.Das chemische Verhalten von Sulfiden wurde mehrfach mit einer ,,Schwefel-dOrbital-Beteiligung" gedeutet3) : So sollen Thioather in kinetisch determinierten nucleophilen Reaktionen wie Kondensationen4), Substitutionens) oder Decarboxylierungens) deshalb urn GroRenordnungen rascher reagieren als ihre Sauerstoffanaloga, weil der Ubergangszustand durch Oktetterweiterung am Schwefel stabilisiert wird. Dem von Longuet-Higgins7) entwickelten ,,pd-Modell" widersprechen jedoch z. B.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.