Lysosomes catabolize and recycle lipids and other biological molecules to maintain cellular
homeostasis in diverse nutrient environments. Lysosomal lipid catabolism relies on the …

Lysosomes critically rely on bis (monoacylglycero) phosphate (BMP) to stimulate lipid
catabolism, cholesterol homeostasis, and lysosomal function. Alterations in BMP levels in …

Ceroid lipofuscinosis neuronal (CLN) genes encode 13 proteins that localize throughout the
endomembrane system to regulate a variety of cellular processes. In humans, mutations in …

Ceroid lipofuscinosis neuronal protein 5 (Cln5) is encoded by the CLN5 gene. The genetic
variants of this gene are associated with the CLN5 form of Batten disease. Recently, the first …

Papain-like cysteine peptidases form a big and highly diverse superfamily of proteins
involved in many important biological functions, such as protein turnover, deubiquitination …

Autophagy is a lysosome-dependent degradation pathway essential for cellular
homeostasis, which decreases with age. However, it is unclear how aging induces …

Sheep with naturally occurring CLN5 and CLN6 forms of neuronal ceroid lipofuscinoses
(Batten disease) share the key clinical features of the human disease and represent an ideal …

The neuronal ceroid lipofuscinoses (NCLs), collectively referred to as Batten disease, are a
group of fatal neurodegenerative disorders that primarily affect children. The etiology of …

Abstract Ceroid lipofuscinosis neuronal 5 (CLN5) and cathepsin D (CTSD) are soluble
lysosomal enzymes that also localize extracellularly. In humans, homozygous mutations in …

Lysosomes are highly dynamic organelles that maintain cellular homeostasis and regulate
fundamental cellular processes by integrating multiple metabolic pathways. Lysosomal ion …