Abstract
Specific pathological hallmarks have been described in amyotrophic lateral sclerosis (ALS), which include motor neuronal loss, Bunina bodies (BBs) and skein like inclusions (SLIs). We investigated the relation between these three lesions in the cervical and lumbar anterior horns and the hypoglossal nuclei of 20 ALS patients and 9 controls using a quantitative light microscopy study. Immunohistochemistry with anti-cystatin C and anti-ubiquitin was used to detect the BBs and SLIs, respectively. A significant relation between the severity of neuronal loss and the proportion of SLI-containing neurons was found in the spinal cord, whereas no relation was found with BBs. We therefore propose that BBs and SLIs participate in two different steps of the cascade leading to neuronal loss.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Aged
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Amyotrophic Lateral Sclerosis / metabolism
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Amyotrophic Lateral Sclerosis / pathology*
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Amyotrophic Lateral Sclerosis / physiopathology
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Brain Stem / metabolism
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Brain Stem / pathology*
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Brain Stem / physiopathology
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Cell Count
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Cell Death / physiology*
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Cystatin C
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Cystatins / metabolism
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Cytoskeleton / metabolism
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Cytoskeleton / pathology*
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Female
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Humans
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Hypoglossal Nerve / metabolism
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Hypoglossal Nerve / pathology
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Hypoglossal Nerve / physiopathology
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Immunohistochemistry
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Inclusion Bodies / metabolism
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Inclusion Bodies / pathology*
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Male
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Middle Aged
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Motor Neurons / metabolism
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Motor Neurons / pathology*
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Spinal Cord / metabolism
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Spinal Cord / pathology*
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Spinal Cord / physiopathology
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Statistics as Topic
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Ubiquitin / metabolism
Substances
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CST3 protein, human
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Cystatin C
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Cystatins
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Ubiquitin