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Type: Journal article
Title: NSF, Unc-18-1, dynamin-1 and HSP90 are inclusion body components in neuronal intranuclear inclusion disease identified by anti-SUMO-1-immunocapture
Author: Pountney, D.
Raftery, M.
Chegini, F.
Blumbergs, P.
Gai, W.
Citation: Acta Neuropathologica, 2008; 116(6):603-614
Publisher: Springer
Issue Date: 2008
ISSN: 0001-6322
Statement of
Dean L. Pountney, Mark J. Raftery, Fariba Chegini, Peter C. Blumbergs, Wei Ping Gai
Abstract: Neuronal intranuclear inclusion disease, a progressive ataxia that may be familial or sporadic, is characterized by numerous neuronal intranuclear inclusion bodies similar to those found in polyglutamine repeat diseases. Previously, we found that the intranuclear inclusion bodies are intensely immunopositive for SUMO-1, a protein which covalently conjugates to other proteins in a similar way to ubiquitin. To identify the SUMO-1-associated proteins in the inclusion bodies, we isolated intranuclear inclusion bodies from fresh, frozen brain tissue of a case with familial neuronal intranuclear inclusion disease and solubilized the proteins. SUMO-1-associated inclusion body proteins were then immunocaptured using an anti-SUMO-1 antibody. The proteins, NSF, dynamin-1 and Unc-18-1 (rbSEC1), involved in membrane trafficking of proteins, and the chaperone HSP90, were identified following anti-SUMO-1-immunocapture by using tandem mass spectrometry and database searching. Immunohistochemistry of brain sections and crude brain homogenates of three cases of familial neuronal intranuclear inclusion disease confirmed the presence of these proteins in intranuclear inclusions.
Keywords: Polyglutamine; Ataxia; Neuronal intranuclear inclusion; SUMO-1
Description: © Springer-Verlag 2008
RMID: 0020083822
DOI: 10.1007/s00401-008-0437-4
Appears in Collections:Pathology publications

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