The neuronal ceroid lipofuscinoses (NCL) are severe neurodegenerative lysosomal storage
disorders of childhood, characterized by accumulation of autofluorescent ceroid …

Neuronal ceroid lipofuscinoses (NCL) comprise a group of inherited lysosomal disorders
with variable age of onset, characterized by lysosomal accumulation of autofluorescent …

Background One of the most important steps taken by Beyond Batten Disease Foundation in
our quest to cure juvenile Batten (CLN3) disease is to understand the State of the Science …

The neuronal ceroid lipofuscinoses (NCL) are a group of disorders defined by shared
clinical and pathological features, including seizures and progressive decline in vision …

Lysosomal membrane proteins act at several crucial steps of the lysosome life cycle,
including lumen acidification, metabolite export, molecular motor recruitment and fusion with …

Loss-of-function mutations in CLN3 are responsible for juvenile-onset neuronal ceroid
lipofuscinosis (JNCL), or Batten disease, which is an incurable lysosomal disease that …

Neuronal ceroid lipofuscinoses are a group of fatal progressive neurodegenerative diseases
predominantly affecting children. Identification of mutations that cause neuronal ceroid …

Numerous lysosomal enzymes and membrane proteins are essential for the degradation of
proteins, lipids, oligosaccharides, and nucleic acids. The CLN3 gene encodes a lysosomal …

Model systems provide an invaluable tool for investigating the molecular mechanisms
underlying the NCLs, devastating neurodegenerative disorders that affect the relatively …

Transition from proliferation to quiescence brings about extensive changes in cellular
behavior and structure. However, the genes that are crucial for establishing and/or …