Intracerebroventricular administration of a modified hexosaminidase ameliorates late-stage neurodegeneration in a GM2 mouse model.

The GM2 gangliosidoses, Tay-Sachs disease and Sandhoff disease, are devastating neurodegenerative disorders caused by β-hexosaminidase A (HexA) deficiency. In the Sandhoff disease mouse model, rescue potential was severely reduced when HexA was introduced after disease onset. Here, we assess the effect of recombinant HexA and HexD3, a newly engineered mimetic of HexA optimized for the treatment of Tay-Sachs disease and Sandhoff disease.

SF1-specific deletion of the energy sensor AMPKγ2 induces obesity.

Objective: AMP-activated protein kinase (AMPK) is a heterotrimer complex consisting of a catalytic α subunit (α1, α2) with a serine/threonine kinase domain, and two regulatory subunits, β (β1, β2) and γ (γ1, γ2, γ3), encoded by different genes. In the hypothalamus, AMPK plays a crucial role in regulating energy balance, including feeding, energy expenditure, peripheral glucose and lipid metabolism. However, most research on hypothalamic AMPK has concentrated on the catalytic subunits AMPKα1 and AMPKα2, with little focus on the regulatory subunits.

Energy poverty and health inequalities in Barcelona: A cross-sectional trends study in the context of COVID-19, energy crisis and climate change, 2016-2021.

The aim of this study was to analyse the time trends in Barcelona before and during the adverse context of COVID-19, energy crisis, and climate change in 1) the energy poverty (EP) prevalence; 2) the association between EP and health and 3) the impact of EP on health, according to the axes of inequality (sex, age, social class, and country of birth). We conducted a cross-sectional trends study using data from the 2016 and 2021 Barcelona Health Survey. This study clearly recognizes that EP continues to be an important public health problem in the context of Barcelona.

Is pancreatitis associated with meglumine antimoniate treatment for canine leishmaniosis? A multicentric prospective study.

Background: Meglumine antimoniate is used to treat canine leishmaniosis. In humans, it has been associated with pancreatitis. Although a few case reports have described acute pancreatitis secondary to antimonial treatment in dogs, some studies have concluded that pancreatitis is not an adverse effect of this medication.

Impairment of lipid homeostasis causes lysosomal accumulation of endogenous protein aggregates through ESCRT disruption.

Protein aggregation increases during aging and is a pathological hallmark of many age-related diseases. Protein homeostasis (proteostasis) depends on a core network of factors directly influencing protein production, folding, trafficking, and degradation. Cellular proteostasis also depends on the overall composition of the proteome and numerous environmental variables.