The DOI matches its Crossref record; 27 of the 27 references it cites were found in a registry or an index.
Skein-like inclusions in the anterior horn cells in motor neuron disease
Abstract
Skein-like inclusions (SLIs) in the anterior horn cells of patients with motor neuron diseases, including familial amyotrophic lateral sclerosis with posterior column degeneration, sporadic lower motor neuron disease and classical amyotrophic lateral sclerosis, were investigated morphologically with hematoxylin and eosin preparations, immunostaining for ubiquitin and immunoelectron microscopy. The SLIs were thready linear or tubular structures which immunostained with antiubiquitin antibodies. They were detected on hematoxylin and eosin preparations as eosinophilic thread-like structures often surrounded by pale areas. SLIs were occasionally present as networks of threads or tubules. Sometimes, they were aggregated and formed larger pale inclusions. Ultrastructurally, the SLIs were bundles of filaments which appeared thicker than neurofilaments. The SLIs tended to have central hollow spaces which were devoid of filaments. When the SLIs were clustered, fuzzy thick filaments were randomly and loosely arranged among the individual SLIs. The SLIs were histologically and ultrastructurally distinct from other inclusions such as Bunina bodies and hyaline inclusions. This unique morphology of SLIs may provide a novel perspective on the degenerative processes of the anterior horn cells in MND.
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“UBQLN2 colocalizes with SQSTM1 and ubiquitin in neuronal aggregates characteristic of both ALS and ALS-FTD [221,224].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [224]
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Cites
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Fine Structural Study of Neurofibrillary Changes in a Family with Amyotrophic Lateral Sclerosis
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Self-citationUbiquitin deposits in anterior horn cells in motor neurone disease
Leigh, P. N., Anderton, B. H., Dodson, A. R., Gallo, J., Swash, M., & Power, D. M. (1988) · Neuroscience Letters · doi:10.1016/0304-3940(88)90081-x · Cited by 279
Familial Amyotrophic Lateral Sclerosis
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Familial Amyotrophic Lateral Sclerosis
Boylan, K. (2015) · Neurologic Clinics · doi:10.1016/j.ncl.2015.07.001 · Cited by 193
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Research works printed but not linked: 2
- Kuroda, 1986, Sporadic amyotrophic lateral sclerosis with intracytoplasmic eosinophilic inclusions, Neurol. Med. (Tokyo), 24, 31
- Mizusawa, 1987, Lower motor neuron disease associated with a focal onion-bulb formation in an anterior spinal root, Neurol. Med. (Tokyo), 26, 309
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Piao, Y., Wakabayashi, K., Kakita, A., Yamada, M., Hayashi, S., Morita, T., Ikuta, F., Oyanagi, K., & Takahashi, H. (2003) · Brain Pathology · doi:10.1111/j.1750-3639.2003.tb00002.x · Cited by 210
Autophagy and ALS: mechanistic insights and therapeutic implications
Chua, J. P., De Calbiac, H., Kabashi, E., & Barmada, S. J. (2022) · Autophagy · doi:10.1080/15548627.2021.1926656 · Cited by 189
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Ubiquitin in Neurodegenerative Diseases
Lowe, J. S., Mayer, R. J., & Landon, M. (1993) · Brain Pathology · doi:10.1111/j.1750-3639.1993.tb00726.x · Cited by 170
Neuronal intermediate filaments and ALS: A new look at an old question
Xiao, S., McLean, J. R., & Robertson, J. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2006.09.003 · Cited by 139
Metadata
- Authors
- Mizusawa, H., Nakamura, H., Wakayama, I., Yen, S.-H. C., & Hirano, A.
- Affiliations
- University of Tsukuba (JP)
- Tottori University (JP)
- Wakayama Medical University (JP)
- Albert Einstein College of Medicine (US)
- Montefiore Medical Center (US)
- ORCID
- Wakayama, Ikuro
- Venue
- Journal of the Neurological Sciences
- Publisher
- Elsevier BV
- Published
- 1991-09
- Volume
- 105
- Issue
- 1
- Pages
- 14-21
- ISSN
- 0022-510X (ISSN-L)
- OpenAlex
- W2056433352
- Cited by
- 63
- References
- 27
- Citations by year
- 2024: 1 · 2023: 1 · 2021: 3 · 2020: 1 · 2019: 1 · 2017: 1 · 2015: 1 · 2014: 1 · 2013: 2 · 2012: 1
- FWCI
- 7.29
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of the Neurological Sciences (publishedVersion)
- PubMed (publishedVersion)
- Funders
- ALS Association
- Ministry of Education
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Keywords
- anterior horn cells, motor neuron disease, amyotrophic lateral sclerosis, posterior column degeneration, lower motor neuron disease, ubiquitin, ultrastructure, neurofilaments, Bunina bodies, neurodegeneration
- MeSH terms
- Adult, Aged, Amyotrophic Lateral Sclerosis, Inclusion Bodies, Female, Humans, Intermediate Filaments, Male, Middle Aged, Motor Neurons, Neuromuscular Diseases, Spinal Cord, Ubiquitins, Microscopy, Immunoelectron
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