Abstract
Rosenthal fibers (RFs) are abnormal inclusions within astrocytes, characteristic of Alexander's disease. We have previously isolated a 22 kd protein component of RFs from Alexander's disease brain. By Western blotting, we detected its equivalent in several rat organs, with the highest level in heart, and in a human astrocytoma cell line (U-373MG). A cDNA library established from U-373MG was screened with an anti-RF protein antibody. A partial cDNA clone encoding the lens protein alpha B-crystallin was isolated. The anti-RF protein antibodies react with lens alpha B-crystallin. Furthermore, the distribution of alpha B-crystallin mRNA in rat organs is consistent with the Western blots. Therefore, alpha B-crystallin is not lens-specific and it can accumulate in large amounts in astrocytes in pathological conditions.
Publication types
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Amino Acid Sequence
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Animals
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Astrocytes / analysis
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Astrocytes / metabolism
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Astrocytes / ultrastructure
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Base Sequence
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Cell Line
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Cloning, Molecular
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Crystallins / analysis
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Crystallins / genetics*
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Crystallins / metabolism
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Diffuse Cerebral Sclerosis of Schilder / metabolism*
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Diffuse Cerebral Sclerosis of Schilder / pathology
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Gene Expression Regulation
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Growth Disorders / metabolism
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Growth Disorders / pathology
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Humans
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Immunohistochemistry
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Inclusion Bodies / ultrastructure
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Intellectual Disability / metabolism*
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Intellectual Disability / pathology
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Intermediate Filament Proteins / genetics
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Intermediate Filament Proteins / immunology
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Intermediate Filament Proteins / metabolism
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Molecular Sequence Data
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Nerve Tissue Proteins / genetics
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Nerve Tissue Proteins / immunology
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Nerve Tissue Proteins / metabolism
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Protein Biosynthesis
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Protein Kinases*
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Rats
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alpha-Crystallin B Chain
Substances
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CRYAB protein, human
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Crystallins
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Intermediate Filament Proteins
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Nerve Tissue Proteins
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alpha-Crystallin B Chain
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Protein Kinases