Abstract
The inferior olivary nucleus (ION) from nine patients with multiple system atrophy was examined with antibodies against alpha-synuclein, ubiquitin, synaptophysin, glial fibrillary acidic protein, the Golgi apparatus (GA)-trans-Golgi network (TGN), and microglia/macrophages. As previously reported, there were neuronal loss, gliosis, and alpha-synuclein-positive cytoplasmic inclusions in neurons and glia. In addition, all neurons with alpha-synuclein-positive cytoplasmic inclusions contained abnormal profiles of the GA and TGN, which were reduced in size and numbers. This finding suggests a relationship between the pathogenetic mechanisms causing inclusion body formation and abnormalities of the GA-TGN. This study is also consistent with the conclusion that lesions of the ION may not always reflect changes of transsynaptic degeneration secondary to Purkinje cell loss.
Publication types
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Comparative Study
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Aged
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Female
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Glial Fibrillary Acidic Protein / metabolism
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Glycoproteins*
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Golgi Apparatus / metabolism
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Golgi Apparatus / pathology*
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Humans
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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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Medulla Oblongata / metabolism
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Medulla Oblongata / pathology*
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Medulla Oblongata / physiopathology
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Membrane Glycoproteins / metabolism
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Membrane Proteins*
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Middle Aged
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Multiple System Atrophy / metabolism
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Multiple System Atrophy / pathology*
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Multiple System Atrophy / physiopathology
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Nerve Tissue Proteins / metabolism
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Neurons / metabolism
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Neurons / pathology*
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Olivary Nucleus / metabolism
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Olivary Nucleus / pathology*
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Olivary Nucleus / physiopathology
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Receptors, Cell Surface / metabolism
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Receptors, Fibroblast Growth Factor
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Retrograde Degeneration / metabolism
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Retrograde Degeneration / pathology*
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Retrograde Degeneration / physiopathology
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Sialoglycoproteins / metabolism
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Synaptophysin / metabolism
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Synucleins
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Ubiquitin / metabolism
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alpha-Synuclein
Substances
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Glial Fibrillary Acidic Protein
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Glycoproteins
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Membrane Glycoproteins
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Membrane Proteins
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Nerve Tissue Proteins
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Receptors, Cell Surface
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Receptors, Fibroblast Growth Factor
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SNCA protein, human
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Sialoglycoproteins
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Synaptophysin
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Synucleins
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TGOLN2 protein, human
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Ubiquitin
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alpha-Synuclein
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cysteine-rich fibroblast growth factor receptor