Neuromuscular junction defects in mice with mutation of dynein heavy chain 1.

Disruptions in axonal transport have been implicated in a wide range of neurodegenerative diseases. Cramping 1 (Cra1/+) and Legs at odd angles (Loa/+) mice, with hypomorphic mutations in the dynein heavy chain 1 gene, which encodes the ATPase of the retrograde motor protein dynein, were originally r...

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Autores principales:	Stephanie L Courchesne, Maria F Pazyra-Murphy, Daniel J Lee, Rosalind A Segal
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2011
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/abbbc5293cf645a0ae11be11ed4deecf
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spelling	oai:doaj.org-article:abbbc5293cf645a0ae11be11ed4deecf2021-11-18T06:59:11ZNeuromuscular junction defects in mice with mutation of dynein heavy chain 1.1932-620310.1371/journal.pone.0016753https://doaj.org/article/abbbc5293cf645a0ae11be11ed4deecf2011-02-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/21346813/?tool=EBIhttps://doaj.org/toc/1932-6203Disruptions in axonal transport have been implicated in a wide range of neurodegenerative diseases. Cramping 1 (Cra1/+) and Legs at odd angles (Loa/+) mice, with hypomorphic mutations in the dynein heavy chain 1 gene, which encodes the ATPase of the retrograde motor protein dynein, were originally reported to exhibit late onset motor neuron disease. Subsequent, conflicting reports suggested that sensory neuron disease without motor neuron loss underlies the phenotypes of Cra1/+ and Loa/+ mice. Here, we present behavioral and anatomical analyses of Cra1/+ mice. We demonstrate that Cra1/+ mice exhibit early onset, stable behavioral deficits, including abnormal hindlimb posturing and decreased grip strength. These deficits do not progress through 24 months of age. No significant loss of primary motor neurons or dorsal root ganglia sensory neurons was observed at ages where the mice exhibited clear symptomatology. Instead, there is a decrease in complexity of neuromuscular junctions. These results indicate that disruption of dynein function in Cra1/+ mice results in abnormal morphology of neuromuscular junctions. The time course of behavioral deficits, as well as the nature of the morphological defects in neuromuscular junctions, suggests that disruption of dynein function in Cra1/+ mice causes a developmental defect in synapse assembly or stabilization.Stephanie L CourchesneMaria F Pazyra-MurphyDaniel J LeeRosalind A SegalPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 6, Iss 2, p e16753 (2011)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Stephanie L Courchesne Maria F Pazyra-Murphy Daniel J Lee Rosalind A Segal Neuromuscular junction defects in mice with mutation of dynein heavy chain 1.
description	Disruptions in axonal transport have been implicated in a wide range of neurodegenerative diseases. Cramping 1 (Cra1/+) and Legs at odd angles (Loa/+) mice, with hypomorphic mutations in the dynein heavy chain 1 gene, which encodes the ATPase of the retrograde motor protein dynein, were originally reported to exhibit late onset motor neuron disease. Subsequent, conflicting reports suggested that sensory neuron disease without motor neuron loss underlies the phenotypes of Cra1/+ and Loa/+ mice. Here, we present behavioral and anatomical analyses of Cra1/+ mice. We demonstrate that Cra1/+ mice exhibit early onset, stable behavioral deficits, including abnormal hindlimb posturing and decreased grip strength. These deficits do not progress through 24 months of age. No significant loss of primary motor neurons or dorsal root ganglia sensory neurons was observed at ages where the mice exhibited clear symptomatology. Instead, there is a decrease in complexity of neuromuscular junctions. These results indicate that disruption of dynein function in Cra1/+ mice results in abnormal morphology of neuromuscular junctions. The time course of behavioral deficits, as well as the nature of the morphological defects in neuromuscular junctions, suggests that disruption of dynein function in Cra1/+ mice causes a developmental defect in synapse assembly or stabilization.
format	article
author	Stephanie L Courchesne Maria F Pazyra-Murphy Daniel J Lee Rosalind A Segal
author_facet	Stephanie L Courchesne Maria F Pazyra-Murphy Daniel J Lee Rosalind A Segal
author_sort	Stephanie L Courchesne
title	Neuromuscular junction defects in mice with mutation of dynein heavy chain 1.
title_short	Neuromuscular junction defects in mice with mutation of dynein heavy chain 1.
title_full	Neuromuscular junction defects in mice with mutation of dynein heavy chain 1.
title_fullStr	Neuromuscular junction defects in mice with mutation of dynein heavy chain 1.
title_full_unstemmed	Neuromuscular junction defects in mice with mutation of dynein heavy chain 1.
title_sort	neuromuscular junction defects in mice with mutation of dynein heavy chain 1.
publisher	Public Library of Science (PLoS)
publishDate	2011
url	https://doaj.org/article/abbbc5293cf645a0ae11be11ed4deecf
work_keys_str_mv	AT stephanielcourchesne neuromuscularjunctiondefectsinmicewithmutationofdyneinheavychain1 AT mariafpazyramurphy neuromuscularjunctiondefectsinmicewithmutationofdyneinheavychain1 AT danieljlee neuromuscularjunctiondefectsinmicewithmutationofdyneinheavychain1 AT rosalindasegal neuromuscularjunctiondefectsinmicewithmutationofdyneinheavychain1
_version_	1718424125256499200

Neuromuscular junction defects in mice with mutation of dynein heavy chain 1.

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