Alpha B-crystallin is expressed in non-lenticular tissues and accumulates in Alexander's disease brain |
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Authors: | T Iwaki A Kume-Iwaki R K Liem J E Goldman |
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Institution: | Department of Pathology, Columbia University College of Physicians and Surgeons, New York State Psychiatric Institute, New York 10032. |
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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. |
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