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

Here, we review the role of oxidative protein modification as a signal for recognition and
degradation of proteins. It was clearly demonstrated that the ATP-and ubiquitin-independent …

The ceroid‐lipofuscinoses (Batten disease) are neurodegenerative inherited lysosomal
storage diseases of children and animals. A common finding is the occurrence of fluorescent …

Despite the identification of a large number of disease-causing genes in recent years, it is
still unclear what disease mechanisms operate in the neuronal ceroid lipofuscinoses (NCLs …

A wide variety of inherited lysosomal hydrolase deficiencies have been reported in animals
and are characterized by accumulation of sphingolipids, glycolipids, oligosaccharides, or …

Pathological studies of mice homozygous for the motor neuron degeneration (Mnd) mutation
show abnormalities similar to those of the human neuronal ceroid lipofuscinoses …

The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are fatal inherited
neurodegenerative diseases characterized by gross brain atrophy, blindness, and …

Neuronal ceroid lipofuscinoses (NCL; Batten disease) are a group of inherited
neurodegenerative diseases primarily affecting children. A common feature across most …

The ceroid lipofuscinoses are a group of neurodegenerative lysosomal storage diseases of
children and animals that are recessively inherited. In diseased individuals fluorescent …

Neuronal ceroid lipofucinoses (NCLs) are a group of severe neurodegenerative disorders
characterized by accumulation of autofluorescent ceroid lipopigment in patients' cells. The …