Springer Nature is making Coronavirus research free. View research | View latest news | Sign up for updates

αB-Crystallin is present in reactive glia in Creutzfeldt-Jakob disease

  • 60 Accesses

  • 150 Citations


α-Crystallin is a major eye lens protein, composed of two types of subunits, αA and αB. The αA subunit is restricted to the lens, but αB-crystallin has recently also been detected in non-lenticular tissues, including the nervous system. With the use of a polyclonal antiserum directed against a synthetic C-terminal peptide of human αB-crystallin, the presence of αB-crystallin could be demonstrated immunohistochemically in astrocytes in the brains of patients with Creutzfeldt-Jakob disease (CJD). Most intensive localization was observed in the spongiotic tissue representing abundant progressively changed astrocytes in CJD. In agematched control brains weak positive reaction was located in individual oligodendroglia cells and subpial astrocytes. Prominent increase of αB-crystallin in pathological glia in CJD may represent a response to stress.

This is a preview of subscription content, log in to check access.


  1. 1.

    Beck E, Daniel PM (1987) Neuropathology of transmissible encephalopathies. In: Prusiner SB, McKinley MP (eds) Prions: novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. Academic Press, San Diego, pp 331–385

  2. 2.

    Bhat SP, Nagineni CN (1989) αB subunit of lens-specific protein α-crystallin is present in other ocular and non-ocular tissues. Biochem Biophys Res Commun 158:319–325

  3. 3.

    De Jong WW, Hoekman WA, Mulders JWM, Bloemendal H (1986) Heat-shock response of the rat lens. J Cell Biol 102:104–111

  4. 4.

    De Jong WW, Hendriks W, Mulders JWM, Bloemendal H (1989) Evolution of eye lens crystallins: the stress connection. Trends Biochem Sci 14:365–368

  5. 5.

    Duguid JR, Rohwer RG, Seed B (1988) Isolation of cDNAs of scrapie-modulated RNAs by subtractive hybridization of a cDNA library. Proc Natl Acad Sci USA 85:5738–5742

  6. 6.

    Friede RL (1962) The cytochemistry of normal and reactive astrocytes. J Neuropathol Exp Neurol 21:471–478

  7. 7.

    Goldman JE, Corbin E (1988) Isolation of a major protein component of Rosenthal fibers. Am J Pathol 130:569–578

  8. 8.

    Hsu SM, Raine L, Fanger H (1981) Use of avidin-biotinperoxidase complex (ABC) in immunoperoxidase techniques: a comparison between ABC and unlabelled antibody (PAP) procedures. J Histochem Cytochem 29:577–580

  9. 9.

    Iwaki T, Kume-Iwaki A, Liem RKH, Goldman JE (1989) αB-crystallin is expressed in non-lenticular tissues and accumulates in Alexander's disease brain. Cell 57:71–78

  10. 10.

    Iwaki T, Kume-Iwaki A, Goldman JE (1990) Cellular distribution of αB-crystallin in non-lenticular tissues. J Histochem Cytochem 38:31–39

  11. 11.

    Kato K, Shinohara H, Kurobe N, Inaguma T, Shimizu K, Ohskima K (1991) Tissue distribution and developmental profiles of immunoreactive αB-crystallin in the rat determined with a sensitive immunoassay system. Acta Biochim Biophys 1074:201–208

  12. 12.

    Klemenz R, Fröhli E, Steiger RH, Schäfer R, Aoyama A (1991) αB-crystallin is a small heat-shock protein. Proc Natl Acad Sci USA 88:3652–3656

  13. 13.

    Kochevar DT, Aucoin MM, Cooper J (1991) Mammalian heat-shock proteins: an overview with a systems perspective. Toxicol Lett 56:243–267

  14. 14.

    Lindquist S, Craig EA (1988) The heat-shock proteins. Annu Rev Genet 22:631–677

  15. 15.

    Lowe J, Landon M, Pike I, Spendlove I, McDermott H, Mayer RJ (1990) Dementia with β-amyloid deposition: involvement of αB-crystallin supports two main diseases. Lancet 336:515–516

  16. 16.

    Nagineni CN, Bhat SP (1988) Maintenance of the synthesis of αB-crystallin and progressive expression of βBp-crystallin in human fetal lens epithelial cells in culture. Dev Biol 130:402–405

  17. 17.

    Nakamura Y, Takeda M, Angelides KJ, Tada K, Hariguchi S, Nishimura T (1991) Assembly, disassembly, and exchange of glial fibrillary acidic protein. Glia 4:101–110

  18. 18.

    Tomokane N, Iwaki T, Tateishi J, Iwaki A, Goldman JE (1991) Rosenthal fibers share epitopes with alpha B-crystallin, glial fibrillary acidic protein, and ubiquitin, but not with vimentin. Immunoelectron microscopy with colloidal gold. Am J Pathol 138:875–885

  19. 19.

    Wistow GJ, Piatigorsky J (1988) Lens crystallins: the evolution and expression of proteins for a highly specialized tissue. Annu Rev Biochem 57:479–504

Download references

Author information

Correspondence to K. Renkawek.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Renkawek, K., de Jong, W.W., Merck, K.B. et al. αB-Crystallin is present in reactive glia in Creutzfeldt-Jakob disease. Acta Neuropathol 83, 324–327 (1992).

Download citation

Key words

  • αB-Crystallin
  • Immunohistochemistry
  • Reactive astrocytes
  • Creutzteldt-Jakob disease
  • Prion disease