NR AGFR
AU Kato,S.; Hirano,A.; Umahara,T.; Llena,J.F.; Herz,F.; Ohama,E.
TI Ultrastructural and immunohistochemical studies on ballooned cortical neurons in Creutzfeldt-Jakob disease: expression of alpha B-crystallin, ubiquitin and stress-response protein 27.
QU Acta Neuropathologica 1992; 84(4): 443-8
PT journal article
AB 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 alpha B-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 synaptophysin, 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 alpha B-crystallin, srp 27 and ubiquitin may be related to the degenerative processes that neurons undergo in CJD.
IN In den meisten geschwollenen Neuronen von 10 Creutzfeldt-Jakob-Patienten wurde die Expression des alpha B-Crystallins und die Ablagerung seiner Aggregate im Cytoplasma festgestellt. Eine ähnliche, wenn auch schwächere Reaktion wurde auch bei Ubiquitin, dem Streßreaktionsprotein 27 und Synaptophysin, jedoch nicht bei Streßreaktionsprotein 72 beobachtet. Die Autoren halten dies für eine Reaktion auf die Degeneration der Neuronen.
MH Aged; Brain/pathology; Cerebral Cortex/*pathology; Creutzfeldt-Jakob Syndrome/immunology/*pathology; Crystallins/*biosynthesis; Female; Heat-Shock Proteins/*biosynthesis; Human; Immunohistochemistry; Male; Middle Age; Neurons/*ultrastructure; Support, Non-U.S. Gov't; Ubiquitins/*biosynthesis
AD Division of Neuropathology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10467.
SP englisch
PO Deutschland