Neuronal ceroid-lipofuscinosis: A clinical and morphological study of 19 patients
β Scribed by Nardocci, Nardo ;Verga, M. Luisa ;Binelli, Simona ;Zorzi, Giovanna ;Angelini, Lucia ;Bugiani, Orso
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 427 KB
- Volume
- 57
- Category
- Article
- ISSN
- 0148-7299
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The neuronal ceroid lipofuscinoses (NCLs) comprise a set of at least 6 distinct human and an unknown number of animal diseases characterized by storage of proteolipids in lysosomes of many cell types. By unknown mechanisms, this accumulation leads to or is associated with severe neuronal and retinal