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Complement activation in acquired and hereditary amyloid neuropathy.
C E Hafer-Macko1, P J Dyck, C L Koski
1Department of Neurology, University of Maryland School of Medicine, Baltimore 21201-1595, USA. cmacko@umaryland.edu
Journal of the Peripheral Nervous System : JPNS
|July 10, 2001
Summary
Complement activation occurs in amyloid neuropathy, contributing to axonal injury. This study found complement activation products on amyloid deposits in both acquired and hereditary forms, suggesting a role in nerve damage.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- The cause of axonal degeneration in amyloid neuropathy remains unclear.
- Amyloidosis can be acquired or hereditary, affecting peripheral nerves.
Purpose of the Study:
- To investigate the role of complement activation in the pathogenesis of axonal degeneration in amyloid neuropathy.
- To compare complement activation patterns in acquired versus hereditary amyloid neuropathy.
Main Methods:
- Immunohistochemistry was used to examine 20 sural nerve biopsies (14 acquired, 6 hereditary amyloid neuropathy).
- Detection of complement activation products and pathways (classical, alternative) was performed.
Main Results:
- Complement activation products were found on and around amyloid deposits in all examined nerve biopsies.
- No significant differences in complement activation were observed between acquired and hereditary amyloid neuropathy.
- Evidence suggests antibody-independent classical pathway activation via C1q binding in hereditary cases.
- Full complement cascade activation, indicated by C5b-9 neoantigen, was detected on amyloid deposits.
Conclusions:
- Complement activation on amyloid deposits is a common feature of amyloid neuropathy, irrespective of acquired or hereditary origin.
- The terminal complement complex (C5b-9) formation in vivo may cause bystander axonal injury.
- Understanding complement's role may offer new therapeutic targets for amyloid neuropathy.