Ubiquitin-positive inclusion in anterior horn cells in subgroups of motor neuron diseases: A comparative study of adult-onset amyotrophic lateral sclerosis, juvenile amyotrophic lateral sclerosis and werdnig-hoffmann disease

Sadayuki Matsumoto, Satoshi Goto, Hirofumi Kusaka, Terukuni Imai, Nobuyuki Murakami, Yoshio Hashizume, Haruo Okazaki, Asao Hirano

Research output: Contribution to journalArticle

40 Citations (Scopus)

Abstract

This report concerns the expression of ubiquitin in anterior horn cells of various subgroups of adult and infantile motor neuron disease (MNDs); immunohistochemical techniques were employed. Ubiquitin-positive skein-like inclusions (SLIs) were found in all cases of adult-onset amyotrophic lateral sclerosis (ALS), including 16 cases with sporadic ALS, two cases of familial ALS with posterior column degeneration and Lewy body-like hyaline incusions (LBHIs), two sporadic ALS cases with LBHIs, and three cases of sporadic ALS with dementia. SLIs were not found in anterior horn cells of 5 cases with Werdnig-Hoffmann disease (WHD). However, granular ubiquitin-positive deposits were seen in ballooned neurons of WHD patients. No ubiquitinated materials were found in the perikarya of two sporadic juvenile ALS patients with basophilic inclusions (BIs), but granular ubiquitin-immunoreactive deposits were occasionally observed in the BIs. These results suggest that ubiquitin-positive SLIs are characteristic features of various forms of adult-onset ALS and that aggregated ubiquitinated granules are characteristic of ballooned neurons of WHD. Ubiquitinated structures and their distribution patterns may reflect degenerative processes of anterior horn cells, and may be useful for classifying subgroups of motor neuron diseases.

Original languageEnglish (US)
Pages (from-to)208-213
Number of pages6
JournalJournal of the Neurological Sciences
Volume115
Issue number2
DOIs
StatePublished - 1993

Fingerprint

Spinal Muscular Atrophies of Childhood
Anterior Horn Cells
Motor Neuron Disease
Amyotrophic Lateral Sclerosis
Ubiquitin
Lewy Bodies
Hyalin
Neurons
Amyotrophic lateral sclerosis 1

Keywords

  • Amyotrophic lateral sclerosis
  • Anterior horn cell
  • Juvenile amyotrophic lateral sclerosis
  • Ubiquitin
  • Werdnig-Hoffmann disease

ASJC Scopus subject areas

  • Aging
  • Clinical Neurology
  • Surgery
  • Neuroscience(all)
  • Developmental Neuroscience
  • Neurology

Cite this

Ubiquitin-positive inclusion in anterior horn cells in subgroups of motor neuron diseases : A comparative study of adult-onset amyotrophic lateral sclerosis, juvenile amyotrophic lateral sclerosis and werdnig-hoffmann disease. / Matsumoto, Sadayuki; Goto, Satoshi; Kusaka, Hirofumi; Imai, Terukuni; Murakami, Nobuyuki; Hashizume, Yoshio; Okazaki, Haruo; Hirano, Asao.

In: Journal of the Neurological Sciences, Vol. 115, No. 2, 1993, p. 208-213.

Research output: Contribution to journalArticle

Matsumoto, Sadayuki ; Goto, Satoshi ; Kusaka, Hirofumi ; Imai, Terukuni ; Murakami, Nobuyuki ; Hashizume, Yoshio ; Okazaki, Haruo ; Hirano, Asao. / Ubiquitin-positive inclusion in anterior horn cells in subgroups of motor neuron diseases : A comparative study of adult-onset amyotrophic lateral sclerosis, juvenile amyotrophic lateral sclerosis and werdnig-hoffmann disease. In: Journal of the Neurological Sciences. 1993 ; Vol. 115, No. 2. pp. 208-213.
@article{f882c95a13ba459086629928c6b71763,
title = "Ubiquitin-positive inclusion in anterior horn cells in subgroups of motor neuron diseases: A comparative study of adult-onset amyotrophic lateral sclerosis, juvenile amyotrophic lateral sclerosis and werdnig-hoffmann disease",
abstract = "This report concerns the expression of ubiquitin in anterior horn cells of various subgroups of adult and infantile motor neuron disease (MNDs); immunohistochemical techniques were employed. Ubiquitin-positive skein-like inclusions (SLIs) were found in all cases of adult-onset amyotrophic lateral sclerosis (ALS), including 16 cases with sporadic ALS, two cases of familial ALS with posterior column degeneration and Lewy body-like hyaline incusions (LBHIs), two sporadic ALS cases with LBHIs, and three cases of sporadic ALS with dementia. SLIs were not found in anterior horn cells of 5 cases with Werdnig-Hoffmann disease (WHD). However, granular ubiquitin-positive deposits were seen in ballooned neurons of WHD patients. No ubiquitinated materials were found in the perikarya of two sporadic juvenile ALS patients with basophilic inclusions (BIs), but granular ubiquitin-immunoreactive deposits were occasionally observed in the BIs. These results suggest that ubiquitin-positive SLIs are characteristic features of various forms of adult-onset ALS and that aggregated ubiquitinated granules are characteristic of ballooned neurons of WHD. Ubiquitinated structures and their distribution patterns may reflect degenerative processes of anterior horn cells, and may be useful for classifying subgroups of motor neuron diseases.",
keywords = "Amyotrophic lateral sclerosis, Anterior horn cell, Juvenile amyotrophic lateral sclerosis, Ubiquitin, Werdnig-Hoffmann disease",
author = "Sadayuki Matsumoto and Satoshi Goto and Hirofumi Kusaka and Terukuni Imai and Nobuyuki Murakami and Yoshio Hashizume and Haruo Okazaki and Asao Hirano",
year = "1993",
doi = "10.1016/0022-510X(93)90226-O",
language = "English (US)",
volume = "115",
pages = "208--213",
journal = "Journal of the Neurological Sciences",
issn = "0022-510X",
publisher = "Elsevier",
number = "2",

}

TY - JOUR

T1 - Ubiquitin-positive inclusion in anterior horn cells in subgroups of motor neuron diseases

T2 - A comparative study of adult-onset amyotrophic lateral sclerosis, juvenile amyotrophic lateral sclerosis and werdnig-hoffmann disease

AU - Matsumoto, Sadayuki

AU - Goto, Satoshi

AU - Kusaka, Hirofumi

AU - Imai, Terukuni

AU - Murakami, Nobuyuki

AU - Hashizume, Yoshio

AU - Okazaki, Haruo

AU - Hirano, Asao

PY - 1993

Y1 - 1993

N2 - This report concerns the expression of ubiquitin in anterior horn cells of various subgroups of adult and infantile motor neuron disease (MNDs); immunohistochemical techniques were employed. Ubiquitin-positive skein-like inclusions (SLIs) were found in all cases of adult-onset amyotrophic lateral sclerosis (ALS), including 16 cases with sporadic ALS, two cases of familial ALS with posterior column degeneration and Lewy body-like hyaline incusions (LBHIs), two sporadic ALS cases with LBHIs, and three cases of sporadic ALS with dementia. SLIs were not found in anterior horn cells of 5 cases with Werdnig-Hoffmann disease (WHD). However, granular ubiquitin-positive deposits were seen in ballooned neurons of WHD patients. No ubiquitinated materials were found in the perikarya of two sporadic juvenile ALS patients with basophilic inclusions (BIs), but granular ubiquitin-immunoreactive deposits were occasionally observed in the BIs. These results suggest that ubiquitin-positive SLIs are characteristic features of various forms of adult-onset ALS and that aggregated ubiquitinated granules are characteristic of ballooned neurons of WHD. Ubiquitinated structures and their distribution patterns may reflect degenerative processes of anterior horn cells, and may be useful for classifying subgroups of motor neuron diseases.

AB - This report concerns the expression of ubiquitin in anterior horn cells of various subgroups of adult and infantile motor neuron disease (MNDs); immunohistochemical techniques were employed. Ubiquitin-positive skein-like inclusions (SLIs) were found in all cases of adult-onset amyotrophic lateral sclerosis (ALS), including 16 cases with sporadic ALS, two cases of familial ALS with posterior column degeneration and Lewy body-like hyaline incusions (LBHIs), two sporadic ALS cases with LBHIs, and three cases of sporadic ALS with dementia. SLIs were not found in anterior horn cells of 5 cases with Werdnig-Hoffmann disease (WHD). However, granular ubiquitin-positive deposits were seen in ballooned neurons of WHD patients. No ubiquitinated materials were found in the perikarya of two sporadic juvenile ALS patients with basophilic inclusions (BIs), but granular ubiquitin-immunoreactive deposits were occasionally observed in the BIs. These results suggest that ubiquitin-positive SLIs are characteristic features of various forms of adult-onset ALS and that aggregated ubiquitinated granules are characteristic of ballooned neurons of WHD. Ubiquitinated structures and their distribution patterns may reflect degenerative processes of anterior horn cells, and may be useful for classifying subgroups of motor neuron diseases.

KW - Amyotrophic lateral sclerosis

KW - Anterior horn cell

KW - Juvenile amyotrophic lateral sclerosis

KW - Ubiquitin

KW - Werdnig-Hoffmann disease

UR - http://www.scopus.com/inward/record.url?scp=0027506412&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0027506412&partnerID=8YFLogxK

U2 - 10.1016/0022-510X(93)90226-O

DO - 10.1016/0022-510X(93)90226-O

M3 - Article

C2 - 8387100

AN - SCOPUS:0027506412

VL - 115

SP - 208

EP - 213

JO - Journal of the Neurological Sciences

JF - Journal of the Neurological Sciences

SN - 0022-510X

IS - 2

ER -