Creutzfeldt-Jakob Disease Research - Mad Cow Disease, Symptoms, Causes, Variants

Creutzfeldt-Jakob Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Creutzfeldt-Jakob Disease, including details on mad cow disease, symptoms, causes, variants.


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Inactivation of amyloid-enhancing factor (AEF): study on experimental murine AA amyloidosis.

Omoto M, Yokota T, Cui D, Hoshii Y, Kawano H, Gondo T, Ishihara T, Kanda T

Department of Neurology and Clinical Neuroscience, Yamaguchi University School of Medicine, 1-1-1 Minamikogushi, Ube City, and Department of Pathology, Kokura Memorial Hospital, Yamaguchi, 755-8505, Japan. omoto-path@umin.ac.jp

It is known that amyloid-enhancing factor (AEF) shortens the preamyloid phase in experimentally induced AA amyloidosis in mice. Because it is reported that AEF serves as both a nidus and a template for amyloid formation, AA amyloidosis may have transmissibility by a prion-like mechanism. It has been shown that amyloid fibrils also have AEF activity, and amyloid fibrils with AEF activity were named fibril-amyloid enhancing factor (F-AEF). In this study, we investigated methods to inactivate the AEF activity. AEF was extracted from the thyroid gland obtained at autopsy of a patient with AA amyloidosis. Before injection into mice, AEF was treated with several methods for inactivation. Of all the tested treatments, 1 N NaOH, 0.1 N NaOH, and autoclaving consistently demonstrated complete inactivation of AEF. Heat treatment led to incomplete inactivation, but 0.01 N NaOH, 0.001 N NaOH, pepsin, trypsin, pronase, and proteinase K treatment had no effect on AEF activity. By analysis with transmission electron microscopy, the AEF preparation contains amyloid fibrils, and a change of ultrastructure was shown after 1 N NaOH, 0.1 N NaOH, and autoclaving treatment. Furthermore, immunoblotting of AEF with antihuman AA antibody revealed that the protein band was scarcely found after autoclaving, 1 N NaOH, and 0.1 N NaOH treatment. Our results suggest that, similar to Creutzfeldt-Jakob disease (CJD), amyloidosis may require chemical or autoclaving decontamination.

Published 18 June 2007 in Med Mol Morphol, 40(2): 88-94.
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Creutzfeldt-Jakob Disease Research Today Archive:

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The Official Patient's Sourcebook on Creutzfeldt-Jakob Disease: A Revised and Updated Directory for the Internet Age