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¥2,700,000 (Direct Cost : ¥2,700,000)
Fiscal Year 1999 : ¥1,000,000 (Direct Cost : ¥1,000,000)
Fiscal Year 1998 : ¥1,700,000 (Direct Cost : ¥1,700,000)
αB-crystallin (αB-C) is a subunit of α-crystallin, a major protein component of the vertebrate lens, and αB-C expression has been reported in extra-lenticular tissues. In normal brains, oligodendrocytes and astrocytes are stained with anti-αB-C antibody, but neurons are not. Since most of previous studies using this antibody have primarily dealt with ballooned neurons, we investigated such αB-C expression in neuronal abnormalities of various neurological diseases.
We examined 73 patients with 29 kinds of neurological diseases and 4 patients with non-neurological diseases. We obtained 4-μm paraffin embadded sections of the spinal cords from all of the patients, in addition to the sections of some areas of the brains involved by neurological diseases. Each section was stained by the routine staining methods and avidin-biotin complex (ABC) method (Vectastain elite, Vector) using anti-αB-C antibody (a gift from Dr. Hiroshi Mori). The anti-αB-C antibody was made by the injection of a synthet
ic peptide from the carboxyl terminal of αB-C to rabbits (Tamaoka et al, 1995).
Frequent immunoreactivity to αB-C included ballooned neurons in corticobasal degeneration (CBD) and Pick's disease, cortical Lewy bodies, ballooned in a new type of familial Parkinson disease (Mizutani et al, 1998), and renurons of the inferior olives with pseudohypertrophy in 2 out of 3 cases. Occasional immunoreactivity was observed in some neurons around cerebral infarcts. Rare immunoreactivity included the anterior hom motor neurons of the spinal cords in amyotrophic lateral sclerosis (ALS), progressive supranuclear palsy (PSP), Machado-Joseph disease, inclusion body myositis, multiple sclerosis, and carcinomatous meningitis, as well as the posterio hom neurons of the spinal cords in ALS, PSP, hepatic myelopathy, inclusion body myositis, and crytococcal meningitis. In the spinal cords, no consistent relationship was observed between the αB-C immunostaining and the kind of diseases, and between the αB-C immunostaining and the distribution of the histopathological lesions. Rare immunoreactivity was also found in the pontine nuclei neurons of multiple system atrophy and CBD. Negative immunoreactivity to αB-C included Pick bodies, brainstem Lewy bodies, Alzheimer's neurofibrillary tangles, and neurons with central chromatolysis.
Our results indicated that αB-C immunostaining to abnormal neurons was selective, and that this staining was useful to detect ballooned neurons and cortical Lewy bodies, and to differentiate balloned neurons from both Pick bodies and neurons with central chromatolysis due to axonal injury. Although neuronal immunoreactivity was observed in other various histopathological lesions, this abnormality was rare, and further studies are needed to clarify the significance of such neuronal immunoreactivity. Less