Diagnosis of late-infantile neuronal ceroid lipofuscinosis: A new sensitive method to assay lysosomal pepstatin-insensitive proteinase activity in human and animal specimens by capillary electrophoresis

Batten disease, or human late-infantile neuronal ceroid lipofuscinosis (LIN CL) is a familiar progressive degenerative disease affecting children, caus ed by a deficiency of a lysosomal proteinase (tripeptidyl peptidase 1, TPP- 1) and characterized by the accumulation of autofluorescent storage bodies in the brain and other tissues of the body. Current methodology used to dia gnose this disease needs to be improved in order to have less invasive tech niques with higher resolution and shorter assay time. In this report, we di scuss the potential merits of micellar electrokinetic chromatography as an excellent tool that requires minute samples but offers high resolution and a short running time for monitoring TPP-1 activity in human and animal spec imens.