Selectivity and Types of Cell Death in the Neuronal Ceroid Lipofuscinoses (NCLs)

Abstract excerpt

Cloning of the individual genes that are mutated in the neuronal ceroid lipofuscinoses (NCLs), or Batten disease, has opened up new avenues of research into the pathogenesis of these fatal autosomal recessive storage disorders. Genetically accurate mouse models have now been generated for each major form of the disorder, together with several variant forms. Ongoing analysis of these mice is revealing significant...

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