Xylose-Configured Cyclophellitols as Selective Inhibitors for Glucocerebrosidase.

Glucocerebrosidase (GBA), a lysosomal retaining β-d-glucosidase, has recently been shown to hydrolyze β-d-xylosides and to transxylosylate cholesterol. Genetic defects in GBA cause the lysosomal storage disorder Gaucher disease (GD), and also constitute a risk factor for developing Parkinson's disease. GBA and other retaining glycosidases can be selectively visualized by activity-based protein profiling (ABPP) using fluorescent probes composed of a cyclophellitol scaffold having a configuration tailored to the targeted glycosidase family.

The Iminosugar AMP-DNM Improves Satiety and Activates Brown Adipose Tissue Through GLP1.

Obesity is taking on worldwide epidemic proportions, yet effective pharmacological agents with long-term efficacy remain unavailable. Previously, we designed the iminosugar N-adamantine-methyloxypentyl-deoxynojirimycin (AMP-DNM), which potently improves glucose homeostasis by lowering excessive glycosphingolipids. Here we show that AMP-DNM promotes satiety and activates brown adipose tissue (BAT) in obese rodents.

Distribution of Nonperfusion and Neovascularization on Ultrawide-Field Fluorescein Angiography in Proliferative Diabetic Retinopathy (RECOVERY Study): Report 1.

Purpose: To explore the distribution of nonperfusion area (NPA) on ultrawide-field fluorescein angiography (UWF FA) in proliferative diabetic retinopathy (PDR) and its relationship with the presence of neovascularization of the optic disc (NVD) and distribution of neovascularization elsewhere (NVE).

Design: Prospective, observational case series.

Methods: Baseline Optos 200Tx UWF FA images of 38 eyes with treatment-naïve early-stage PDR from the RECOVERY (NCT02863354) study were stereographically projected at the Doheny Image Reading Center.

Comparison of Subjective Assessment and Precise Quantitative Assessment of Lesion Distribution in Diabetic Retinopathy.

Importance: Predominantly peripheral disease in eyes with nonproliferative diabetic retinopathy (DR) is suggested as a potential strong risk factor for progression to proliferative disease. However, the reliability and optimal method for the assessment of lesion distribution are still uncertain.

Objective: To compare agreement between subjective assessment and precise quantification of lesion burden in ultrawidefield (UWF) images of eyes with DR.