The neuronal ceroid lipofuscinoses Wies 3

  title={The neuronal ceroid lipofuscinoses Wies 3},
  author={awa Grajkowska and bieta Czarnowska and Tomasz Kmie{\ae} and Maciej Pronicki},
The neuronal lipofuscinoses (NCLs) are described as a complex of inherited neurodegenerative disorders associated with intralysosomal accumulation of lipopigment in neuronal and extraneuronal cells. Morphologic pathology in NCL is marked by two processes: degeneration and loss of neuronal cells of central nervous system, resulting in atrophy of the cerebrum and cerebellum and widespread accumulation of autofluorescent lysosomal lipopigments of varying ultrastructure, the demonstration of which… CONTINUE READING
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