Alpha B-crystallin is expressed in non-lenticular tissues and accumulates in Alexander's disease brain

Cell. 1989 Apr 7;57(1):71-8. doi: 10.1016/0092-8674(89)90173-6.


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

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Astrocytes / analysis
  • Astrocytes / metabolism
  • Astrocytes / ultrastructure
  • Base Sequence
  • Cell Line
  • Cloning, Molecular
  • Crystallins / analysis
  • Crystallins / genetics*
  • Crystallins / metabolism
  • Diffuse Cerebral Sclerosis of Schilder / metabolism*
  • Diffuse Cerebral Sclerosis of Schilder / pathology
  • Gene Expression Regulation
  • Growth Disorders / metabolism
  • Growth Disorders / pathology
  • Humans
  • Immunohistochemistry
  • Inclusion Bodies / ultrastructure
  • Intellectual Disability / metabolism*
  • Intellectual Disability / pathology
  • Intermediate Filament Proteins / genetics
  • Intermediate Filament Proteins / immunology
  • Intermediate Filament Proteins / metabolism
  • Molecular Sequence Data
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / immunology
  • Nerve Tissue Proteins / metabolism
  • Protein Biosynthesis
  • Protein Kinases*
  • Rats
  • alpha-Crystallin B Chain


  • CRYAB protein, human
  • Crystallins
  • Intermediate Filament Proteins
  • Nerve Tissue Proteins
  • alpha-Crystallin B Chain
  • Protein Kinases

Associated data

  • GENBANK/M24906