Apical dendrite degeneration, a novel cellular pathology for Betz cells in ALS

Barlay Genç, Javier H Jara, Amiko K.B. Lagrimas, Peter Pytel, Raymond P. Roos, Marek-Marsel Mesulam, Changiz Geula, Eileen H Bigio, Pembe Hande Ozdinler*

*Corresponding author for this work

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Apical dendrites of Betz cells are important sites for the integration of cortical input, however their health has not been fully assessed in ALS patients. We investigated the primary motor cortices isolated from post-mortem normal control subjects, patients with familial ALS (fALS), sporadic ALS (sALS), ALS with frontotemporal dementia (FTD-ALS), and Alzheimer's disease (AD), and found profound apical dendrite degeneration of Betz cells in both fALS and sALS, as well as FTD-ALS patients. In contrast, Betz cells of AD patients and normal controls retain cellular integrity in the motor cortex, and CA1 pyramidal neurons show abnormalities predominantly within their soma, rather than the apical dendrite. In line with extensive vacuolation and cytoarchitectural disintegration, the numbers of synapses were also significantly reduced only in ALS patients. Our findings indicate apical dendrite degeneration as a novel cellular pathology that distinguishes ALS and further support the importance of cortical dysfunction for disease pathology.

Original languageEnglish (US)
Article number41765
JournalScientific reports
Volume7
DOIs
StatePublished - Feb 6 2017

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Dendrites
Pathology
Motor Cortex
Alzheimer Disease
Frontotemporal Dementia
Pyramidal Cells
Carisoprodol
Synapses
Health

ASJC Scopus subject areas

  • General

Cite this

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title = "Apical dendrite degeneration, a novel cellular pathology for Betz cells in ALS",
abstract = "Apical dendrites of Betz cells are important sites for the integration of cortical input, however their health has not been fully assessed in ALS patients. We investigated the primary motor cortices isolated from post-mortem normal control subjects, patients with familial ALS (fALS), sporadic ALS (sALS), ALS with frontotemporal dementia (FTD-ALS), and Alzheimer's disease (AD), and found profound apical dendrite degeneration of Betz cells in both fALS and sALS, as well as FTD-ALS patients. In contrast, Betz cells of AD patients and normal controls retain cellular integrity in the motor cortex, and CA1 pyramidal neurons show abnormalities predominantly within their soma, rather than the apical dendrite. In line with extensive vacuolation and cytoarchitectural disintegration, the numbers of synapses were also significantly reduced only in ALS patients. Our findings indicate apical dendrite degeneration as a novel cellular pathology that distinguishes ALS and further support the importance of cortical dysfunction for disease pathology.",
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Apical dendrite degeneration, a novel cellular pathology for Betz cells in ALS. / Genç, Barlay; Jara, Javier H; Lagrimas, Amiko K.B.; Pytel, Peter; Roos, Raymond P.; Mesulam, Marek-Marsel; Geula, Changiz; Bigio, Eileen H; Ozdinler, Pembe Hande.

In: Scientific reports, Vol. 7, 41765, 06.02.2017.

Research output: Contribution to journalArticle

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AU - Genç, Barlay

AU - Jara, Javier H

AU - Lagrimas, Amiko K.B.

AU - Pytel, Peter

AU - Roos, Raymond P.

AU - Mesulam, Marek-Marsel

AU - Geula, Changiz

AU - Bigio, Eileen H

AU - Ozdinler, Pembe Hande

PY - 2017/2/6

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AB - Apical dendrites of Betz cells are important sites for the integration of cortical input, however their health has not been fully assessed in ALS patients. We investigated the primary motor cortices isolated from post-mortem normal control subjects, patients with familial ALS (fALS), sporadic ALS (sALS), ALS with frontotemporal dementia (FTD-ALS), and Alzheimer's disease (AD), and found profound apical dendrite degeneration of Betz cells in both fALS and sALS, as well as FTD-ALS patients. In contrast, Betz cells of AD patients and normal controls retain cellular integrity in the motor cortex, and CA1 pyramidal neurons show abnormalities predominantly within their soma, rather than the apical dendrite. In line with extensive vacuolation and cytoarchitectural disintegration, the numbers of synapses were also significantly reduced only in ALS patients. Our findings indicate apical dendrite degeneration as a novel cellular pathology that distinguishes ALS and further support the importance of cortical dysfunction for disease pathology.

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