Pathology of the inferior olivary nucleus in patients with multiple system atrophy.
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.
Sakurai
Atsushi
A
Department of Neurology, Gunma University School of Medicine, 3-39-15, Showa-machi, Maebashi, 371-8511, Japan. atsushis@dan.wind.ne.jp
Okamoto
Koichi
K
Yaguchi
Masamitsu
M
Fujita
Yukio
Y
Mizuno
Yuji
Y
Nakazato
Yoichi
Y
Gonatas
Nicholas K
NK
D000368:Aged; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006023:Glycoproteins; D006056:Golgi Apparatus / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008526:Medulla Oblongata / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D008562:Membrane Glycoproteins / Q000378:metabolism; D008565:Membrane Proteins; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D009419:Nerve Tissue Proteins / Q000378:metabolism; D009474:Neurons / Q000378:metabolism / Q000473:pathology*; D009847:Olivary Nucleus / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D011956:Receptors, Cell Surface / Q000378:metabolism; D017468:Receptors, Fibroblast Growth Factor; D012183:Retrograde Degeneration / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D012795:Sialoglycoproteins / Q000378:metabolism; D016708:Synaptophysin / Q000378:metabolism; D051843:Synucleins; D025801:Ubiquitin / Q000378:metabolism; D051844:alpha-Synuclein
D005904:Glial Fibrillary Acidic Protein; D006023:Glycoproteins; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D011956:Receptors, Cell Surface; D017468:Receptors, Fibroblast Growth Factor; C497604:SNCA protein, human; D012795:Sialoglycoproteins; D016708:Synaptophysin; D051843:Synucleins; C072237:TGOLN2 protein, human; D025801:Ubiquitin; D051844:alpha-Synuclein; C086711:cysteine-rich fibroblast growth factor receptor