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Immunocytochemical and lectin-binding studies on Lafora bodies
P D Lewis1, D J Evans, B Shambayati
1Department of Histopathology, Royal Postgraduate Medical School, Hammermith Hospital, London, UK.
Clinical Neuropathology
|January 1, 1990
Summary
Researchers investigated Lafora bodies in Lafora disease using intermediate filament and lectin antibodies. Lafora bodies contain neurofilaments and desmin, with mannose contributing to their carbohydrate composition.
Area of Science:
- Neuropathology
- Biochemistry
Background:
- Lafora disease is a rare, fatal neurodegenerative disorder.
- Lafora bodies are characteristic intracellular inclusions found in neurons and other cells.
Purpose of the Study:
- To characterize the molecular composition of Lafora bodies.
- To investigate the role of intermediate filaments and carbohydrates in Lafora body formation.
Main Methods:
- Immunohistochemistry using antibodies against neurofilaments (160 KD, 200 KD) and desmin.
- Lectins (concanavalin A) and carbohydrate inhibition assays to analyze carbohydrate content.
Main Results:
- Lafora bodies showed positive staining for 160 KD and 200 KD neurofilaments and desmin.
- Concanavalin A staining patterns indicated that mannose is a component of the non-glucose carbohydrate fraction.
Conclusions:
- Lafora bodies are composed of specific intermediate filament proteins.
- Mannose is a significant contributor to the carbohydrate content of Lafora bodies, offering insights into their structure.