Ubiquitinated filamentous inclusions in spinal cord of patients with motor neuron disease

The ultrastructural localization of ubiquitin (Ubq) in spinal cord of 2 cases of amyotrophic lateral sclerosis was studied, using immunoperoxidase and immunogold labeling on Vibratome sections or on sections cut from paraffin blocks. Two different types of ubiquitinated cytoplasmic inclusions were o...

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Bibliographic Details
Published inNeuroscience letters Vol. 114; no. 1; p. 5
Main Authors Migheli, A, Autilio-Gambetti, L, Gambetti, P, Mocellini, C, Vigliani, M C, Schiffer, D
Format Journal Article
LanguageEnglish
Published Ireland 22.06.1990
Subjects
Amyotrophic Lateral Sclerosis - pathology
Antibodies, Monoclonal
Cytoskeleton - ultrastructure
Humans
Immune Sera
Immunoenzyme Techniques
Inclusion Bodies - ultrastructure
Intermediate Filaments - ultrastructure
Motor Neurons - ultrastructure
Neuromuscular Diseases - pathology
Spinal Cord - pathology
Spinal Cord - ultrastructure
Ubiquitins - analysis
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Summary:The ultrastructural localization of ubiquitin (Ubq) in spinal cord of 2 cases of amyotrophic lateral sclerosis was studied, using immunoperoxidase and immunogold labeling on Vibratome sections or on sections cut from paraffin blocks. Two different types of ubiquitinated cytoplasmic inclusions were observed: (1) bundles composed of 10-15 nm filaments; (2) small rounded bodies without a limiting membrane. A panel of antibodies to neurofilaments (NFs) did not stain the ubiquitinated bundles, and decorated only axonal swellings. Ubq-positive filamentous deposits could be identified with inclusions previously described ultrastructurally. The absence of staining with antibodies to NFs might be due to abnormalities of the filaments, possibly because of a dysfunction of the ubq proteolytic system.
ISSN:0304-3940
DOI:10.1016/0304-3940(90)90419-A