Long-Term Monitoring of Cardiac Involvement under Migalastat Treatment Using Magnetic Resonance Tomography in Fabry Disease.

Fabry cardiomyopathy is characterized by left ventricular hypertrophy, myocardial fibrosis, arrhythmia, and premature death. Treatment with migalastat, an oral pharmacological chaperone, was associated with a stabilization of cardiac biomarkers and a reduction in left ventricular mass index, as measured by echocardiography. A recent study, using cardiac magnetic resonance (CMR) as the gold standard, found a stable course of myocardial involvement after 18 months of treatment with migalastat.

Sequence-independent activation of photocycloadditions using two colours of light.

We exploit two reactive chromophores to establish sequence-independent photochemical activation, employing -methyl benzaldehyde (MBA) and ,-(dimethylamino)pyrene aryl tetrazole (APAT) with -(2-hydroxy)ethyl maleimide (HEM), without any additives. Critically, the order of the irradiation sequence is irrelevant, as the shorter wavelength does not activate the higher wavelength activated species. Therefore, full sequence-independent λ-orthogonality is achieved through differences in both the reaction quantum yields ( and ) and wavelength-dependent reactivity profiles of the employed chromophores.

Green-light induced cycloadditions.

We introduce a red-shifted tetrazole that is able to undergo efficient nitrile imine-mediated tetrazole-ene cycloaddition (NITEC) under blue and green light irradiation. We provide a detailed wavelength-dependent reactivity map, and employ a number of LEDs for high-conversion small molecule and polymer end-group modification.

Photocycloadditions in disparate chemical environments.

We elucidate the wavelength dependence of a photocycloaddition by (i) accessing action plots dependent on the reactivity relative to the number of absorbed photons, (ii) establishing the effect on substrate concentration on photochemical reactivity and (iii) determining wavelength-dependent reactivity as a function of the solvent environment, comparing acetonitrile with dimethyl sulfoxide.