Abstract |
Batten disease, or human late-infantile neuronal ceroid lipofuscinosis (LINCL) is a familiar progressive degenerative disease affecting children, caused by a deficiency of a lysosomal proteinase ( tripeptidyl peptidase I, TPP-I) and characterized by the accumulation of autofluorescent storage bodies in the brain and other tissues of the body. Current methodology used to diagnose this disease needs to be improved in order to have less invasive techniques with higher resolution and shorter assay time. In this report, we discuss 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-I activity in human and animal specimens.
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Authors | S Viglio, E Marchi, K Wisniewski, B Casado, G Cetta, P Iadarola |
Journal | Electrophoresis
(Electrophoresis)
Vol. 22
Issue 11
Pg. 2343-50
(Jul 2001)
ISSN: 0173-0835 [Print] Germany |
PMID | 11504071
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Micelles
- Tpp1 protein, mouse
- Tpp1 protein, rat
- Tripeptidyl-Peptidase 1
- Endopeptidases
- Serine Proteases
- Aminopeptidases
- Dipeptidyl-Peptidases and Tripeptidyl-Peptidases
- TPP1 protein, human
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Topics |
- Aminopeptidases
- Animals
- Blood Platelets
(enzymology)
- Cattle
- Chromatography
(methods)
- Dipeptidyl-Peptidases and Tripeptidyl-Peptidases
- Electrophoresis, Capillary
(methods)
- Endopeptidases
(analysis, blood, deficiency)
- Fibroblasts
(enzymology)
- Humans
- Infant
- Leukocytes
(enzymology)
- Lysosomes
(enzymology)
- Mice
- Micelles
- Neuronal Ceroid-Lipofuscinoses
(diagnosis, enzymology)
- Rats
- Serine Proteases
- Tripeptidyl-Peptidase 1
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