Alpha B-crystallin is expressed in non-lenticular tissues and accumulates in Alexander's disease brain.
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.
Iwaki
T
T
Department of Pathology, Columbia University College of Physicians and Surgeons, New York State Psychiatric Institute, New York 10032.
Kume-Iwaki
A
A
Liem
R K
RK
Goldman
J E
JE
D000595:Amino Acid Sequence; D000818:Animals; D001253:Astrocytes / Q000032:analysis / Q000378:metabolism / Q000648:ultrastructure; D001483:Base Sequence; D002460:Cell Line; D003001:Cloning, Molecular; D003459:Crystallins / Q000032:analysis / Q000235:genetics* / Q000378:metabolism; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000378:metabolism* / Q000473:pathology; D005786:Gene Expression Regulation; D006130:Growth Disorders / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000648:ultrastructure; D008607:Intellectual Disability / Q000378:metabolism* / Q000473:pathology; D007381:Intermediate Filament Proteins / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D008969:Molecular Sequence Data; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D014176:Protein Biosynthesis; D011494:Protein Kinases; D051381:Rats; D038203:alpha-Crystallin B Chain
C059215:CRYAB protein, human; D003459:Crystallins; D007381:Intermediate Filament Proteins; D009419:Nerve Tissue Proteins; D038203:alpha-Crystallin B Chain; D011494:Protein Kinases