Tensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.

Complexins (Cplxs) are small, soluble, regulatory proteins that bind reversibly to the SNARE complex and modulate synaptic vesicle release. Cplx1 knockout mice (Cplx1(-/-)) have the earliest known onset of ataxia seen in a mouse model, although hitherto no histopathology has been described in these...

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Autores principales:	Catherine Kielar, Stephen J Sawiak, Paloma Navarro Negredo, Desmond H Y Tse, A Jennifer Morton
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2012
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/179f3e7b512c4be5a1fa83a8a54d700f
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spelling	oai:doaj.org-article:179f3e7b512c4be5a1fa83a8a54d700f2021-11-18T07:26:23ZTensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.1932-620310.1371/journal.pone.0032636https://doaj.org/article/179f3e7b512c4be5a1fa83a8a54d700f2012-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/22393426/?tool=EBIhttps://doaj.org/toc/1932-6203Complexins (Cplxs) are small, soluble, regulatory proteins that bind reversibly to the SNARE complex and modulate synaptic vesicle release. Cplx1 knockout mice (Cplx1(-/-)) have the earliest known onset of ataxia seen in a mouse model, although hitherto no histopathology has been described in these mice. Nevertheless, the profound neurological phenotype displayed by Cplx1(-/-) mutants suggests that significant functional abnormalities must be present in these animals. In this study, MRI was used to automatically detect regions where structural differences were not obvious when using a traditional histological approach. Tensor-based morphometry of Cplx1(-/-) mouse brains showed selective volume loss from the thalamus and cerebellum. Stereological analysis of Cplx1(-/-) and Cplx1(+/+) mice brain slices confirmed the volume loss in the thalamus as well as loss in some lobules of the cerebellum. Finally, stereology was used to show that there was loss of cerebellar granule cells in Cplx1(-/-) mice when compared to Cplx1(+/+) animals. Our study is the first to describe pathological changes in Cplx1(-/-) mouse brain. We suggest that the ataxia in Cplx1(-/-) mice is likely to be due to pathological changes in both cerebellum and thalamus. Reduced levels of Cplx proteins have been reported in brains of patients with neurodegenerative diseases. Therefore, understanding the effects of Cplx depletion in brains from Cplx1(-/-) mice may also shed light on the mechanisms underlying pathophysiology in disorders in which loss of Cplx1 occurs.Catherine KielarStephen J SawiakPaloma Navarro NegredoDesmond H Y TseA Jennifer MortonPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 7, Iss 2, p e32636 (2012)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Catherine Kielar Stephen J Sawiak Paloma Navarro Negredo Desmond H Y Tse A Jennifer Morton Tensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.
description	Complexins (Cplxs) are small, soluble, regulatory proteins that bind reversibly to the SNARE complex and modulate synaptic vesicle release. Cplx1 knockout mice (Cplx1(-/-)) have the earliest known onset of ataxia seen in a mouse model, although hitherto no histopathology has been described in these mice. Nevertheless, the profound neurological phenotype displayed by Cplx1(-/-) mutants suggests that significant functional abnormalities must be present in these animals. In this study, MRI was used to automatically detect regions where structural differences were not obvious when using a traditional histological approach. Tensor-based morphometry of Cplx1(-/-) mouse brains showed selective volume loss from the thalamus and cerebellum. Stereological analysis of Cplx1(-/-) and Cplx1(+/+) mice brain slices confirmed the volume loss in the thalamus as well as loss in some lobules of the cerebellum. Finally, stereology was used to show that there was loss of cerebellar granule cells in Cplx1(-/-) mice when compared to Cplx1(+/+) animals. Our study is the first to describe pathological changes in Cplx1(-/-) mouse brain. We suggest that the ataxia in Cplx1(-/-) mice is likely to be due to pathological changes in both cerebellum and thalamus. Reduced levels of Cplx proteins have been reported in brains of patients with neurodegenerative diseases. Therefore, understanding the effects of Cplx depletion in brains from Cplx1(-/-) mice may also shed light on the mechanisms underlying pathophysiology in disorders in which loss of Cplx1 occurs.
format	article
author	Catherine Kielar Stephen J Sawiak Paloma Navarro Negredo Desmond H Y Tse A Jennifer Morton
author_facet	Catherine Kielar Stephen J Sawiak Paloma Navarro Negredo Desmond H Y Tse A Jennifer Morton
author_sort	Catherine Kielar
title	Tensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.
title_short	Tensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.
title_full	Tensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.
title_fullStr	Tensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.
title_full_unstemmed	Tensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.
title_sort	tensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.
publisher	Public Library of Science (PLoS)
publishDate	2012
url	https://doaj.org/article/179f3e7b512c4be5a1fa83a8a54d700f
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Tensor-based morphometry and stereology reveal brain pathology in the complexin1 knockout mouse.

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