Motor outcomes in patients with infantile and juvenile Pompe disease: Lessons from neurophysiological findings.

In Infantile Onset Pompe Disease (IOPD), enzyme replacement therapy (ERT) may improve survival, cardiac function, and motor development. However, even with early enzyme replacement therapy, some patients experienced poor response to ERT and abnormal motor milestones that could be due to motor neuron involvement. In this long-term retrospective study, we analyzed concomitant clinical motor outcomes and electroneuromyography (ENMG) findings in patients with IOPD and Juvenile Onset Pompe Disease (JOPD).

Nickel Poisoning of a Cracking Catalyst Unravelled by Single-Particle X-ray Fluorescence-Diffraction-Absorption Tomography.

Ni contamination from crude oil in the fluid catalytic cracking (FCC) process is one of the primary sources of catalyst deactivation, thereby promoting dehydrogenation-hydrogenation and speeding up coke growth. Herein, single-particle X-ray fluorescence, diffraction and absorption (μXRF-μXRD-μXAS) tomography is used in combination with confocal fluorescence microscopy (CFM) after thiophene staining to spatially resolve Ni interaction with catalyst components and study zeolite degradation, including the processes of dealumination and Brønsted acid sites distribution changes. The comparison between a Ni-lean particle, exposed to hydrotreated feedstock, and a Ni-rich one, exposed to non-hydrotreated feedstock, reveals a preferential interaction of Ni, found in co-localization with Fe, with the γ-Al O matrix, leading to the formation of spinel-type hotspots.

Denaturation level of DNA-Pt complexes evidenced by Tb3+ fluorescence enhancement and electric dichroism.

The Tb3+ fluorescence is greatly enhanced, as a result of binding of various platinum coordination complexes to DNA, as compared to native DNA. The largest enhancement is observed for cis-Pt(NH3)2Cl2 but the fluorescence intensity does not however reach the level attained for thermally denatured DNA. Diethylenetriamine-Pt(II) produces very little increase of Tb3+ fluorescence.