Acta Neuropathologica

, Volume 84, Issue 4, pp 443–448 | Cite as

Ultrastructural and immunohistochemical studies on ballooned cortical neurons in Creutzfeldt-Jakob disease: expression of αB-crystallin, ubiquitin and stress-response protein 27

  • S. Kato
  • A. Hirano
  • T. Umahara
  • J. F. Llena
  • F. Herz
  • E. Ohama
Regular Papers


This report concerns ultrastructural and immunohistochemical studies on ballooned neurons of ten patients with Creutzfeldt-Jakob disease (CJD). While abundant ballooned neurons and severe white matter degeneration was seen in six Japanese cases, only occasional ballooned neurons and no white matter degeneration was observed in four cases from the files of Montefiore Medical Center. Ultrastructurally, the ballooned neurons contained granule-coated fibrils of 25 to 40 nm in width and 10-nm neurofilaments. The immunohistochemical studies revealed that most ballooned neurons expressed αB-crystallin, with deposits of reaction products observed in the cytoplasm. A similar intracellular staining pattern was also seen with the antibody to phosphorylated neurofilament proteins (pNFP). Although the proportion of stained ballooned neurons was less, a positive reaction was also observed with antibodies against ubiquitin, stress-response protein 27 (srp 27) and synptophysin, but not with an antibody to srp 72. Our findings suggest that expression of pNFP and synaptophysin by ballooned neurons may reflect axonal impairment and that the presence of αB-crystallin, srp 27 and ubiquitin may be related to the degenerative processes that neurons undergo in CJD.

Key words

Creutzfeldt-Jakob disease Ballooned neurons Ultrastructure αB-Crystallin Stress-response proteins 


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Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • S. Kato
    • 1
  • A. Hirano
    • 1
  • T. Umahara
    • 1
  • J. F. Llena
    • 1
  • F. Herz
    • 2
  • E. Ohama
    • 3
  1. 1.Division of Neuropathology, Montefiore Medical CenterAlbert Einstein College of MedicineBronxUSA
  2. 2.Department of Pathology, Montefiore Medical CenterAlbert Einstein College of MedicineBronxUSA
  3. 3.Division of Neuropathology, Institute of Neurological SciencesTottori University School of MedicineYonagoJapan

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