On the Composition of Autofluorescent Accumulation Products: Ceroid and Lipofuscin
Ceroid was isolated post mortem from the brain of a patient, 29 years old, with the juvenile type of neuronal ceroidlipofuscinosis (NCL). Lipofuscins were isolated post mortem from the brains of individuals, 65 years old and 78 years old, free of neurological disorders. The lipofuscins were pooled. The isolated ceroid and the pooled isolated lipofuscins were fractionated with chloroform/methanol 2:1 (v/v) (fraction A), chloroform/methanol/water 10:10:3 (v/v/v) (fraction B), leaving behind a residue (fraction C).
Biochemical analyses of these fractions, of both lipopigments, were performed with special attention to the occurrence of carbohydrates, dolichol and dolichol derivatives.
KeywordsEnzymatic Conversion Lysosomal Storage Disease High Performance Thin Layer Chromatography Mannose Residue Neuronal Ceroidlipofuscinosis
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