The mitochondrial permeability transition (PT) is a permeability increase of the inner
mitochondrial membrane mediated by a channel, the permeability transition pore (PTP) …

Treatment of the neuronal ceroid lipofuscinoses, also known as Batten disease, is at the start
of a new era because of diagnostic and therapeutic advances relevant to this group of …

The lysosome plays a pivotal role between catabolic and anabolic processes as the nexus
for signalling pathways responsive to a variety of factors, such as growth, nutrient …

The neuronal ceroid-lipofuscinoses (NCLs), collectively also called Batten disease,
constitute one of the most common groups of inherited neurodegenerative disorders in …

Neuronal ceroid lipofuscinosis (NCLs) is a group of inherited neurodegenerative lysosomal
storage diseases that together represent the most common cause of dementia in children …

Lysosomal storage diseases (LSDs) include approximately 70 distinct disorders that
collectively account for 14% of all inherited metabolic diseases. LSDs are caused by …

Frontotemporal dementia (FTD) is a neurodegenerative disease, leading to behavioral
changes and language difficulties. Heterozygous loss-of-function mutations in progranulin …

The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative
lysosomal storage disorders (LSDs), traditionally grouped together based on shared clinical …

Phosphorylated and non-phosphorylated forms of the F 0 F 1-ATPase subunit c from rat liver
mitochondria (RLM) were purified and their effect on the opening of the permeability …

Neuronal ceroid lipofuscinosis type 2 (CLN2 disease) is an autosomal recessive condition
caused by variants in the TPP1 gene, leading to deficient activity of the lysosomal enzyme …