niemann-pick diseases/glutathione

Niemann-Pick disease, type C1 (NPC1) is a fatal, neurodegenerative disorder for which there is no definitive therapy. In NPC1, a pathological cascade including neuroinflammation, oxidative stress and neuronal apoptosis likely contribute to the clinical phenotype. While the genetic cause of NPC1 is

Niemann-Pick disease type C (NPC) is an autosomal recessive neurovisceral lipid storage disease caused by a loss of NPCI function, which results in perturbation of intracellular cholesterol transport. In BALB/c npc(nih) mice, the murine ortholog of NPCI gene is mutated. In NPC mouse, hypomyelination

BACKGROUND Niemann-Pick disease type C (NPC) is caused by defects in cholesterol efflux from lysosomes due to mutations of genes coding for NPC1 and NPC2 proteins. As a result, massive accumulation of unesterified cholesterol in late endosomes/lysosomes is observed. At the level of the organism

The epididymis plays a major role in the acquisition of sperm fertility. In order to shed light on specific features of epididymal function in mammalian species, we characterized the luminal proteins (luminal proteome) and secreted proteins (secretome) in the bovine epididymis. We identified 172