Ubiquitinated alpha B-crystallin in glial cytoplasmic inclusions from the brain of a patient with multiple system atrophy.
Glial cytoplasmic inclusions (GCIs) have been observed in oligodendroglia-like cells, specifically in the brains of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome). We have investigated GCIs from brains of patients with multiple system atrophy biochemically and immunochemically. While most GCIs have been reported positive for both ubiquitin and alpha B-crystallin in immunocytochemical studies, the components of GCIs have not been identified biochemically. GCI-bearing cells were partially purified from the OPCA brain by sieving with nylon meshes and discontinuous sucrose density gradient centrifugation. The fraction containing GCI-bearing cells was also found to contain a 32 kDa and a 40 kDa protein, both of which were specifically recognized by anti-ubiquitin and anti-alpha B-crystallin antibodies, neither of which was found in the same fraction derived from control brain. These immunochemical results suggest that ubiquitinated alpha B-crystallin is present in GCIs from the brains of patients with multiple system atrophy.
Tamaoka
A
A
Department of Neurology, University of Tsukuba, Ibaraki-ken, Japan.
Mizusawa
H
H
Mori
H
H
Shoji
S
S
D000595:Amino Acid Sequence; D001284:Atrophy; D001923:Brain Chemistry / Q000502:physiology*; D003459:Crystallins / Q000737:chemistry*; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000378:metabolism; D008969:Molecular Sequence Data; D009410:Nerve Degeneration / Q000502:physiology*; D009849:Olivopontocerebellar Atrophies / Q000378:metabolism* / Q000473:pathology; D012791:Shy-Drager Syndrome / Q000378:metabolism* / Q000473:pathology; D014452:Ubiquitins / Q000032:analysis* / Q000737:chemistry
D003459:Crystallins; D014452:Ubiquitins