Cholesterol is an essential component of mammalian cell membranes and a precursor for
crucial signaling molecules. The brain contains the highest level of cholesterol in the body …

Abstract Purpose of Review This review explores the interplay among metabolic dysfunction,
oxidative stress, inflammation, and fibrosis in Fabry disease, focusing on their potential …

Mitochondria and lysosomes are two organelles that carry out both signaling and metabolic
roles in cells. Recent evidence has shown that mitochondria and lysosomes are dependent …

N-acetylcysteine (NAC) is widely used as a mucolytic agent and as an antidote to
paracetamol overdose. NAC serves as a precursor of cysteine and stimulates the synthesis …

Lysosomal storage diseases (LSD) are inborn errors of metabolism resulting in multisystem
disease. Central nervous system involvement, often with progressive neurodegeneration …

Mitochondrial dysfunction has been recognized as a key player in neurodegenerative
diseases, including Alzheimer's disease (AD) and Niemann–Pick type C (NPC) disease …

Abstract The Niemann–Pick type C1 (NPC1) protein regulates the transport of cholesterol
and fatty acids from late endosomes/lysosomes and has a central role in maintaining lipid …

Mitochondrial dysfunction has been recognised a major contributory factor to the
pathophysiology of a number of lysosomal storage disorders (LSDs). The cause of …

Glutathione (GSH) is a thiol that plays a significant role in nutrient metabolism, antioxidant
defense and the regulation of cellular events. GSH deficiency is related to variety of …

Biomedical research aims to understand the molecular mechanisms causing human
diseases and to develop curative therapies. So far, these goals have been achieved for a …