Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue

Autores
Müller Igaz, Lionel Ivan; Quintá, Héctor Ramiro
Año de publicación
2021
Idioma
inglés
Tipo de recurso
documento de conferencia
Estado
versión publicada
Descripción
The neurodegenerative diseases amyotrophic lateralsclerosis (ALS) and frontotemporal dementia (FTD) represent two ends of one spectrum disorder, termed ALS/FTD. These incurable pathologies are now classified as ?TDP-43 proteinopathies?,since mislocalization and aggregation of the nuclear protein TDP-43 arehallmark features of most cases. A main feature ofALS/FTD is degeneration of the corticospinaltract (CST), composed of axons of upper motor neurons, being the main motorpathway involved in voluntary movement. We are using a novel approach,combining a cost-effective unsectioned brain/spinal cord clearing technique,fluoroRuby staining, one-photon confocal microscopy and 3D reconstruction tostudy the morphological changes in the CST of TDP-43 transgenic (TG) mice. Wehave previously shown in mice that inducible overexpression of a cytoplasmic (ΔNLS) form of TDP-43 in forebrain neurons evokesneuropathological and behavioural changes that recapitulate several features ofTDP-43 proteinopathies. Our preliminary results showed proper and consistenttracer delivery, with similar number of labelled cortical neurons in controland TG mice. TDP-43-ΔNLS expression decreased the length of cortical apicalprocesses and the number of cervical axons. Remarkably, suppression of TG expression(displaying reversible motor phenotypes) led to an increase in cervical axonalbranching. These studies will help to elucidate the mechanisms underlying themotor phenotypes in ALS/FTD.
Fil: Müller Igaz, Lionel Ivan. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Fisiología y Biofísica Bernardo Houssay. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Fisiología y Biofísica Bernardo Houssay; Argentina
Fil: Quintá, Héctor Ramiro. Hospital Aleman. Laboratorio de Medicina Experimental; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
XXXIV Congreso Anual de la Sociedad Argentina de Investigación en Neurociencias
Argentina
Sociedad Argentina de Investigación en Neurociencias
Materia
TDP-43
DEGENERATION
TRANSGENIC MICE
ALS/FTD
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/194222
Acceder
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network_name_str CONICET Digital (CONICET)
spelling Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissueMüller Igaz, Lionel IvanQuintá, Héctor RamiroTDP-43DEGENERATIONTRANSGENIC MICEALS/FTDhttps://purl.org/becyt/ford/3.1https://purl.org/becyt/ford/3The neurodegenerative diseases amyotrophic lateralsclerosis (ALS) and frontotemporal dementia (FTD) represent two ends of one spectrum disorder, termed ALS/FTD. These incurable pathologies are now classified as ?TDP-43 proteinopathies?,since mislocalization and aggregation of the nuclear protein TDP-43 arehallmark features of most cases. A main feature ofALS/FTD is degeneration of the corticospinaltract (CST), composed of axons of upper motor neurons, being the main motorpathway involved in voluntary movement. We are using a novel approach,combining a cost-effective unsectioned brain/spinal cord clearing technique,fluoroRuby staining, one-photon confocal microscopy and 3D reconstruction tostudy the morphological changes in the CST of TDP-43 transgenic (TG) mice. Wehave previously shown in mice that inducible overexpression of a cytoplasmic (ΔNLS) form of TDP-43 in forebrain neurons evokesneuropathological and behavioural changes that recapitulate several features ofTDP-43 proteinopathies. Our preliminary results showed proper and consistenttracer delivery, with similar number of labelled cortical neurons in controland TG mice. TDP-43-ΔNLS expression decreased the length of cortical apicalprocesses and the number of cervical axons. Remarkably, suppression of TG expression(displaying reversible motor phenotypes) led to an increase in cervical axonalbranching. These studies will help to elucidate the mechanisms underlying themotor phenotypes in ALS/FTD.Fil: Müller Igaz, Lionel Ivan. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Fisiología y Biofísica Bernardo Houssay. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Fisiología y Biofísica Bernardo Houssay; ArgentinaFil: Quintá, Héctor Ramiro. Hospital Aleman. Laboratorio de Medicina Experimental; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaXXXIV Congreso Anual de la Sociedad Argentina de Investigación en NeurocienciasArgentinaSociedad Argentina de Investigación en NeurocienciasSociedad Argentina de Investigación en Neurociencias2021info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/conferenceObjectCongresoBookhttp://purl.org/coar/resource_type/c_5794info:ar-repo/semantics/documentoDeConferenciaapplication/pdfapplication/mswordapplication/pdfhttp://hdl.handle.net/11336/194222Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue; XXXIV Congreso Anual de la Sociedad Argentina de Investigación en Neurociencias; Argentina; 2021; 1-1CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://saneurociencias.org.ar/congresos-san-2/Nacionalinfo:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-29T10:23:43Zoai:ri.conicet.gov.ar:11336/194222instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-29 10:23:44.236CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue
title Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue
spellingShingle Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue
Müller Igaz, Lionel Ivan
TDP-43
DEGENERATION
TRANSGENIC MICE
ALS/FTD
title_short Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue
title_full Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue
title_fullStr Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue
title_full_unstemmed Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue
title_sort Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue
dc.creator.none.fl_str_mv Müller Igaz, Lionel Ivan
Quintá, Héctor Ramiro
author Müller Igaz, Lionel Ivan
author_facet Müller Igaz, Lionel Ivan
Quintá, Héctor Ramiro
author_role author
author2 Quintá, Héctor Ramiro
author2_role author
dc.subject.none.fl_str_mv TDP-43
DEGENERATION
TRANSGENIC MICE
ALS/FTD
topic TDP-43
DEGENERATION
TRANSGENIC MICE
ALS/FTD
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.1
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv The neurodegenerative diseases amyotrophic lateralsclerosis (ALS) and frontotemporal dementia (FTD) represent two ends of one spectrum disorder, termed ALS/FTD. These incurable pathologies are now classified as ?TDP-43 proteinopathies?,since mislocalization and aggregation of the nuclear protein TDP-43 arehallmark features of most cases. A main feature ofALS/FTD is degeneration of the corticospinaltract (CST), composed of axons of upper motor neurons, being the main motorpathway involved in voluntary movement. We are using a novel approach,combining a cost-effective unsectioned brain/spinal cord clearing technique,fluoroRuby staining, one-photon confocal microscopy and 3D reconstruction tostudy the morphological changes in the CST of TDP-43 transgenic (TG) mice. Wehave previously shown in mice that inducible overexpression of a cytoplasmic (ΔNLS) form of TDP-43 in forebrain neurons evokesneuropathological and behavioural changes that recapitulate several features ofTDP-43 proteinopathies. Our preliminary results showed proper and consistenttracer delivery, with similar number of labelled cortical neurons in controland TG mice. TDP-43-ΔNLS expression decreased the length of cortical apicalprocesses and the number of cervical axons. Remarkably, suppression of TG expression(displaying reversible motor phenotypes) led to an increase in cervical axonalbranching. These studies will help to elucidate the mechanisms underlying themotor phenotypes in ALS/FTD.
Fil: Müller Igaz, Lionel Ivan. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Fisiología y Biofísica Bernardo Houssay. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Fisiología y Biofísica Bernardo Houssay; Argentina
Fil: Quintá, Héctor Ramiro. Hospital Aleman. Laboratorio de Medicina Experimental; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
XXXIV Congreso Anual de la Sociedad Argentina de Investigación en Neurociencias
Argentina
Sociedad Argentina de Investigación en Neurociencias
description The neurodegenerative diseases amyotrophic lateralsclerosis (ALS) and frontotemporal dementia (FTD) represent two ends of one spectrum disorder, termed ALS/FTD. These incurable pathologies are now classified as ?TDP-43 proteinopathies?,since mislocalization and aggregation of the nuclear protein TDP-43 arehallmark features of most cases. A main feature ofALS/FTD is degeneration of the corticospinaltract (CST), composed of axons of upper motor neurons, being the main motorpathway involved in voluntary movement. We are using a novel approach,combining a cost-effective unsectioned brain/spinal cord clearing technique,fluoroRuby staining, one-photon confocal microscopy and 3D reconstruction tostudy the morphological changes in the CST of TDP-43 transgenic (TG) mice. Wehave previously shown in mice that inducible overexpression of a cytoplasmic (ΔNLS) form of TDP-43 in forebrain neurons evokesneuropathological and behavioural changes that recapitulate several features ofTDP-43 proteinopathies. Our preliminary results showed proper and consistenttracer delivery, with similar number of labelled cortical neurons in controland TG mice. TDP-43-ΔNLS expression decreased the length of cortical apicalprocesses and the number of cervical axons. Remarkably, suppression of TG expression(displaying reversible motor phenotypes) led to an increase in cervical axonalbranching. These studies will help to elucidate the mechanisms underlying themotor phenotypes in ALS/FTD.
publishDate 2021
dc.date.none.fl_str_mv 2021
dc.type.none.fl_str_mv info:eu-repo/semantics/publishedVersion
info:eu-repo/semantics/conferenceObject
Congreso
Book
http://purl.org/coar/resource_type/c_5794
info:ar-repo/semantics/documentoDeConferencia
status_str publishedVersion
format conferenceObject
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/194222
Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue; XXXIV Congreso Anual de la Sociedad Argentina de Investigación en Neurociencias; Argentina; 2021; 1-1
CONICET Digital
CONICET
url http://hdl.handle.net/11336/194222
identifier_str_mv Aassessing degeneration of corticospinal tracts in a tdp-43 transgenic mouse model of als/ftd: application of 3d reconstruction in cleared tissue; XXXIV Congreso Anual de la Sociedad Argentina de Investigación en Neurociencias; Argentina; 2021; 1-1
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://saneurociencias.org.ar/congresos-san-2/
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/msword
application/pdf
dc.coverage.none.fl_str_mv Nacional
dc.publisher.none.fl_str_mv Sociedad Argentina de Investigación en Neurociencias
publisher.none.fl_str_mv Sociedad Argentina de Investigación en Neurociencias
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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