Publication: Recent advances in research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutations: Neuronal Lewy body-like hyaline inclusions
and astrocytic hyaline inclusions
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Date
1999
Authors
Kato, S. ; Saito, M. ; Hirano, A. ; Ohama, E.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Amyotrophic lateral sclerosis (ALS) is a
progressive neurodegenerative disease that primarily
involves the motor neuron system. Of all patients with
ALS, approximately 5%-10% of them are familial and
most of the others are sporadic. Superoxide dismutase 1
(SOD1) gene mutations are shown to be associated with
about 20% of familial ALS (FALS) patients. FALS is
neuropathologically classified into two subtypes:
classical FALS in which degeneration is restricted to
only motor neurons and FALS which is characterized by
the degeneration of the posterior column in addition to
the lesion of the motor neuron system. The neuronal
Lewy body-like hyaline inclusion (LBHI) is a
characteristic neuropathological marker of mutant
SOD1-linked FALS with posterior column involvement.
Inclusions similar to the neuronal LBHIs have been
discovered in astrocytes in certain patients with FALS
exhibiting SODl gene mutations. The purpose of this
review is to discuss the novel neuropathological
significance of the astrocytic hyaline inclusions (Ast-
HIS) and neuronal LBHIs in brain tissues from
individuals with the posterior-column-involvement-type
FALS with SODl gene mutations. In hematoxylin and
eosin preparations, both Ast-HIS and neuronal LBHIs are
eosinophilic inclusions and sometimes show eosinophilic
cores with paler peripheral halos. Immunohistochemically,
both inclusions are intensely positive for
SODl. At the ultrastructural level, both inclusions
consist of approximately 15-25 nm-sized granule-coated
fibrils and granular materials. Immunoelectron
microscopically, these abnormal granule-coated fibrils
and granular materials are positive for SOD1. Therefore the FALS disease process originating from SODl gene
mutations occurs in astrocytes as well as neurons and is
involved in the formation of both inclusions.
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