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- Title
Cytochemical analysis of storage materials in cultured skin fibroblasts from patients with I-cell disease.
- Abstract
The article discusses the identification of the accumulated substances in cultured I-cell disease fibroblasts by cytochemical methods, such as double-staining with a monoclonal antibody to lysosome-associated membrane protein-1 (LAMP-1). It was found that patient's cells were stained with the antibody to GM2 gangliosided, confirming lysosomal storage. Moreover, it is believed that a metabolic gridlock represent a common mechanism of disease, resulting from enzyme deficiencies.
- Subjects
CYTOCHEMICAL bioassay; STAINS &; staining (Microscopy); MONOCLONAL antibodies; LYSOSOMES; MEMBRANE proteins; GANGLIOSIDOSES; ENZYMES
- Publication
Current Medical Literature: Lysosomal Storage Disease, 2007, Vol 7, Issue 2, p53
- ISSN
1473-0901
- Publication type
Article