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Fine Structural Study of Neurofibrillary Changes in a Family with Amyotrophic Lateral Sclerosis
Abstract
Lewy body-like hyaline inclusions in the soma and swollen, cord-like cell processes are characteristic alterations of the anterior horn cells in familial amyotrophic lateral sclerosis (ALS) with posterior column and spinocerebellar tract involvement. A fine structural analysis of these two structures has been performed in two brothers from a family ("C" family) previously described by Kurland and Mulder in 1955. The perikaryal hyaline inclusions consisted of accumulations of randomly oriented neurofilaments interspersed with thick linear densities associated with granular material. Some of the accumulations showed a central condensation. Cord-like, swollen neuronal processes were composed, for the most part, of numerous neurofilaments arranged parallel to the long axes. Dense structures were sometimes observed within the large bundles of filaments. They were composed of ill-defined dense, granular and fibrillar material associated with scattered vesicles and mitochondria. These dense areas were sometimes surrounded by various amounts of fine filaments, approximately 5 nm in diameter.
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Citations
Cited by
Caughey, B., & Lansbury, P. T. (2003) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.26.010302.081142 · Cited by 1,650
UNRAVELING THE MECHANISMS INVOLVED IN MOTOR NEURON DEGENERATION IN ALS
Bruijn, L. I., Miller, T. M., & Cleveland, D. W. (2004) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.27.070203.144244 · Cited by 1,185
Oxidative stress in ALS: A mechanism of neurodegeneration and a therapeutic target
Barber, S. C., Mead, R. J., & Shaw, P. J. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2006.03.008 · Cited by 474
Julien, J.-P. (2001) · Cell · doi:10.1016/s0092-8674(01)00244-6 · Cited by 434
Metadata
- Authors
- HIRANO, A., NAKANO, I., KURLAND, L. T., MULDER, D. W., HOLLEY, P. W., & SACCOMANNO, G.
- Venue
- Journal of Neuropathology and Experimental Neurology
- Publisher
- Oxford University Press (OUP)
- Published
- 1984-09
- Volume
- 43
- Issue
- 5
- Pages
- 471-480
- ISSN
- 0022-3069 (ISSN-L)
- OpenAlex
- W2017222498
- Cited by
- 309
- References
- 0
- FWCI
- 3.77
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Fine Structural Study of Neurofibrillary Changes in a Family with Amyotrophic Lateral Sclerosis. https://app.cochat.ai/papers/10.1097/00005072-198409000-00002
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- HIRANO, A., NAKANO, I., KURLAND, L. T., MULDER, D. W., HOLLEY, P. W., & SACCOMANNO, G. (1984). Fine Structural Study of Neurofibrillary Changes in a Family with Amyotrophic Lateral Sclerosis. Journal of Neuropathology and Experimental Neurology. https://doi.org/10.1097/00005072-198409000-00002
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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