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Neuronal Ceroid Lipofuscinosis in Dogs

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Neuronal Ceroid Lipofuscinosis (NCL) is the name for a family of eight genetically separate neurodegenerative disorders in dogs that result from excessive accumulation of lipopigments (lipofuscin) in the body's tissues. The disease results from intraneuronal accumulations of ceroid-lipofuscin granules. It has been related to primary cerebellar disease in the dog and can result in cerebellar atrophy.


Unfortunately the prognosis for lysosomal storage diseases in dogs is not good. For the majority of these disorders affected dogs must be euthanized due to progressive worsening neurological dysfunction. Despite the poor outlook Neuronal Ceroid Lipofuscinosis (NCL) bone marrow transplants and lysosomal enzyme replacement therapy have been successful in some animal cases. Gene transfer therapy is also being actively investigated and will hopefully be available in the future.

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