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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
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Complement activation at the motor end-plates in amyotrophic lateral sclerosis
Nawal Bahia El Idrissi1, Sanne Bosch1, Valeria Ramaglia1
1Department of Genome Analysis, Academic Medical Center, Amsterdam, 1105 AZ, The Netherlands.
Journal of Neuroinflammation
|April 9, 2016
Summary
Complement activation occurs at the motor end-plate in amyotrophic lateral sclerosis (ALS) patients, potentially preceding neurodegeneration. This immune response may play a role in ALS progression.
Area of Science:
- Neuroimmunology
- Neuropathology
Background:
- Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease lacking effective therapies.
- Innate immune system components, including complement, are implicated in ALS pathology.
- Complement activation at the motor end-plate may precede neurodegeneration in ALS models.
Purpose of the Study:
- To investigate the role of complement activation and its regulators at the motor end-plate in human ALS.
- To analyze post-mortem tissues from ALS patients for complement deposition.
Main Methods:
- Analysis of post-mortem intercostal muscle biopsies from ALS (n=11) and control (n=6) donors.
- Immunofluorescence and immunohistochemistry used to detect C1q, membrane attack complex (MAC), CD55, and CD59 at motor end-plates.
Main Results:
- Complement activation products (C1q, MAC) and regulators (CD55, CD59) were deposited on motor end-plates in ALS patients but not controls.
- C1q co-localized with neurofilament in ALS intercostal muscle.
- MAC deposition was observed on innervated motor end-plates in ALS patients.
Conclusions:
- Complement activation products are present on innervated motor end-plates in human ALS.
- Complement activation may precede motor end-plate denervation in ALS.
- Complement represents a potential modifier of ALS disease processes.
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