Rapid quantitation of globotriaosylceramide in human plasma and urine: a potential application for monitoring enzyme replacement therapy in Anderson-Fabry disease

A method for measuring globotriaosylceramide (Gb3, or GL3) levels in plasma and urine of humans affected by Anderson-Fabry disease has been developed. The analyses are performed using flow injection analysis-electrospray ionization-tandem mass spectrometry (FIA-ESI-MS/MS). The method is rapid, sensitive and hence suitable for high-throughput analyses, requiring only a simple 50-fold dilution for the preparation of plasma and urine samples. The detection of the analytes of interest was achieved using a triple quadrupole instrument operating in the multiple reaction monitoring mode. The linearity of the calibration standard responses, the intra- and inter-assay precision, the accuracy and the detection limit of the method were evaluated. The proposed method allows a rapid and accurate assessment of globotriaosylceramide in biological samples. Data obtained from healthy volunteers and Anderson-Fabry affected subjects suggest a potential role for this technique in monitoring the effectiveness of Anderson-Fabry disease therapy. The results obtained in two actual cases treated with enzyme replacement therapy are reported and discussed.     

Poly(ethylene glycol)-supported tetrahydroxyphenyl porphyrin: a convenient,recyclable catalyst for photooxidation reactions

[reaction: see text] A new poly(ethylene glycol)-supported porphyrin has been prepared and its ability as a promoter in photooxidation reactions has been studied. The PEG-supported catalyst exhibits high activity, comparable to that of a nonanchored sensitizer, and it is easily removable by filtration from the reaction mixture. The polymer-bound porphyrin has been recycled up to six times with no loss of chemical and stereochemical efficiency.     

Theoretical evaluation of zirconia and hafnia as gate oxides for si microelectronics

Parameters determining the performance of the crystalline oxides zirconia (ZrO2) and hafnia (HfO2) as gate insulators in nanometric Si electronics are estimated via ab initio calculations of the energetics, dielectric properties, and band alignment of bulk and thin-film oxides on Si (001). With their large dielectric constants, stable and low-formation-energy interfaces, large valence offsets, and reasonable (though not optimal) conduction offsets (electron injection barriers), zirconia and hafnia appear to have considerable potential as gate oxides for Si electronics.     

On equilibrium solutions of diffusion equations with nonlinear boundary conditions

The existence or the nonexistence of nonconstant stable equilibrium solutions for a diffusion equation with nonlinear Neumann boundary conditions is studied. We prove the nonexistence of nonconstant stable equilibria when the nonlinearity has a small Lipschitz constant or a second derivative of constant sign or either when the domain is a ball. We construct an example of existence for a connected domain with several disconnected boundary components.This work is partially supported by the project PB91-0497-C02-02 of the DGICYT (Spain).     

The topology of two-dimensional real algebraic varieties

Sunto È noto che ogni spazio analitico reale è localmente omeomorfo al cono su un poliedro con caratteristica di Eulero-Poincaré pari. Si dimostra che questa condizione è anche sufficiente affinchè un poliedro (compatto) di dimensione due P sia omeomorfo ad una varietà algebrica reale affine P. Segue inoltre dalla costruzione che la P ottenuta ha, in un certo senso, un insieme di singolarità algebriche minimale, compatibilmente con la topologia di P.

---

---

The authors are members of the G.N.S.A.G.A.     

Lepton spectrum in semileptonic b-decay

We present some results from the computation of the charged lepton energy distribution in the decay b → c + $\text{l}$^? + $\text{ν}$ taking into account first order QCD corrections.     

Thermodynamic basis for viscoelastic and non-isothermal fracture mechanics

Stability criteria for crack propagation have been analysed which are based on the second law of thermodynamics. The isothermal case has been studied both for elastic and viscoelastic materials. The non-isothermal criteria have been considered with particular attention to a fast adiabatic loading process.The thermodynamic constitutive equations which are demanded and their relationships with the elastic energy have been analyzed.     

A macrocyclic ligand able to bind gallium(III) by preorganized pendant arms; coordination and kinetic studies

The equilibria and kinetics of the binding of gallium(III) to 4-(N),10-(N)-bis[2-(3-hydroxo-2-oxo-2-H-pyridine-1-y1)acetamido]-1,7-dimethyl-1,4,7,10-tetraazacyclododecane (L) were investigated in acidic medium at ionic strength 1 M (NaClO4). Spectrophotometric titrations in the UV region revealed that L is able to bind Ga3+ also at high H+ concentration. The kinetic (stopped-flow) experiments are interpreted on the basis of three parallel reaction paths (i) M3+ + H2L2+ = M(H2L)5+ where M(H2L)5+ is in a steady state, (ii) M(OH)2+ + H2L2+ = M(HL)4+ + H2O and (iii) M(OH)2+ + HL+ = ML3+ + H2O. The first-order rate constants for conversion of the outer-sphere into the inner-sphere complexes are similar to those of the Ga(III)/tropolone system which is known to react according to the dissociative Id mechanism and to the relevant rate constants for water exchange at the metal ion. The effects of pH on the UV-Vis absorption, fluorescence emission properties and NMR spectral features on the Ga(III)/L system were also investigated. Spectrophotometric titrations in the UV region reveal that, in acid medium the prevailing species is M(HL)4+ whereas the chelate ML3+ prevails for [H+] < 0.01 M. The results indicate metal coordination at the oxygen atoms of the 3-hydroxo-2-oxopyridine residues.