Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina

Autores
Di Mauro, Giuliana Constanza; De Ambrosi, Bruno; Uchitel, Osvaldo Daniel; Mazzone, Graciela Luján
Año de publicación
2021
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Amyotrophic Lateral Sclerosis (ALS) is a degenerative disorder characterized by ongoing loss of motoneurons. The etiology of the sporadic ALS formit is thought to be related to immune-mediated motoneuron degeneration and death. The present study was designed to describe the effect of serumfactors derived from sporadic ALS patients on mouse spinal cord preparations in vitro. Sera from patients with sporadic ALS were collected and usedfor immunofluorescence analysis to investigate their effects on neuronal survival and microgliosis. Our experiments demonstrated that 5 h applicationof serum factors (derived from three ALS patients) labeled with human IgG secondary antibody were localized to ventral spinal neurons identified withthe NeuN marker. Moreover, a significant reduction in the number of ventral NeuN positive cells was observed with serum from a patient who sufferedfrom upper motor neuron signs as criteria for ALS diagnosis (20% less compared to sham, spinal cord preparations without serum). No change inmicroglia number was found after exposure to ALS sera, although in two cases a significant decrease in microglial branch length was observed (28-32%).Microglia morphology showed increased number of end points and branches with serum from the patient with upper motor neuron symptoms. Theseobservations were absent after control sera. Our data indicate that spinal cord cultures can be a useful model to further characterize the pathologicalprocesses of sporadic ALS and the immune mechanism as previously suggested in vivo. Future studies are needed to unveil the molecular mechanismsunderlying this preferential targeting of neurons and microglia by ALS serum.
Fil: Di Mauro, Giuliana Constanza. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Fisiología, Biología Molecular y Neurociencias. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Fisiología, Biología Molecular y Neurociencias; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigación en Biomedicina de Buenos Aires - Instituto Partner de la Sociedad Max Planck; Argentina
Fil: De Ambrosi, Bruno. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; Argentina
Fil: Uchitel, Osvaldo Daniel. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Fisiología, Biología Molecular y Neurociencias. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Fisiología, Biología Molecular y Neurociencias; Argentina. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; Argentina
Fil: Mazzone, Graciela Luján. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; Argentina. Universidad Austral. Facultad de Ciencias Biomédicas. Instituto de Investigaciones en Medicina Traslacional. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigaciones en Medicina Traslacional; Argentina
Materia
AMYOTROPHIC LATERAL SCLEROSIS
AUTOIMMUNITY
MICROGLIAL ACTIVATION
NEURON LABELING
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:11336/185057
Acceder
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oai_identifier_str oai:ri.conicet.gov.ar:11336/185057
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Case Report of Neuron-Binding IgGs in ALS Patient Serum in ArgentinaDi Mauro, Giuliana ConstanzaDe Ambrosi, BrunoUchitel, Osvaldo DanielMazzone, Graciela LujánAMYOTROPHIC LATERAL SCLEROSISAUTOIMMUNITYMICROGLIAL ACTIVATIONNEURON LABELINGhttps://purl.org/becyt/ford/1.6https://purl.org/becyt/ford/1Amyotrophic Lateral Sclerosis (ALS) is a degenerative disorder characterized by ongoing loss of motoneurons. The etiology of the sporadic ALS formit is thought to be related to immune-mediated motoneuron degeneration and death. The present study was designed to describe the effect of serumfactors derived from sporadic ALS patients on mouse spinal cord preparations in vitro. Sera from patients with sporadic ALS were collected and usedfor immunofluorescence analysis to investigate their effects on neuronal survival and microgliosis. Our experiments demonstrated that 5 h applicationof serum factors (derived from three ALS patients) labeled with human IgG secondary antibody were localized to ventral spinal neurons identified withthe NeuN marker. Moreover, a significant reduction in the number of ventral NeuN positive cells was observed with serum from a patient who sufferedfrom upper motor neuron signs as criteria for ALS diagnosis (20% less compared to sham, spinal cord preparations without serum). No change inmicroglia number was found after exposure to ALS sera, although in two cases a significant decrease in microglial branch length was observed (28-32%).Microglia morphology showed increased number of end points and branches with serum from the patient with upper motor neuron symptoms. Theseobservations were absent after control sera. Our data indicate that spinal cord cultures can be a useful model to further characterize the pathologicalprocesses of sporadic ALS and the immune mechanism as previously suggested in vivo. Future studies are needed to unveil the molecular mechanismsunderlying this preferential targeting of neurons and microglia by ALS serum.Fil: Di Mauro, Giuliana Constanza. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Fisiología, Biología Molecular y Neurociencias. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Fisiología, Biología Molecular y Neurociencias; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigación en Biomedicina de Buenos Aires - Instituto Partner de la Sociedad Max Planck; ArgentinaFil: De Ambrosi, Bruno. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; ArgentinaFil: Uchitel, Osvaldo Daniel. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Fisiología, Biología Molecular y Neurociencias. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Fisiología, Biología Molecular y Neurociencias; Argentina. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; ArgentinaFil: Mazzone, Graciela Luján. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; Argentina. Universidad Austral. Facultad de Ciencias Biomédicas. Instituto de Investigaciones en Medicina Traslacional. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigaciones en Medicina Traslacional; ArgentinaResearch Open2021-07info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/185057Di Mauro, Giuliana Constanza; De Ambrosi, Bruno; Uchitel, Osvaldo Daniel; Mazzone, Graciela Luján; Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina; Research Open; Journal of Neurology and Neurocritical Care; 4; 2; 7-2021; 1-52771-9065CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://researchopenworld.com/case-report-of-neuron-binding-iggs-in-als-patient-serum-in-argentina/info:eu-repo/semantics/altIdentifier/doi/10.31038/JNNC.2021423info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-10-22T11:47:30Zoai:ri.conicet.gov.ar:11336/185057instacron: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:34982025-10-22 11:47:30.826CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina
title Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina
spellingShingle Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina
Di Mauro, Giuliana Constanza
AMYOTROPHIC LATERAL SCLEROSIS
AUTOIMMUNITY
MICROGLIAL ACTIVATION
NEURON LABELING
title_short Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina
title_full Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina
title_fullStr Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina
title_full_unstemmed Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina
title_sort Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina
dc.creator.none.fl_str_mv Di Mauro, Giuliana Constanza
De Ambrosi, Bruno
Uchitel, Osvaldo Daniel
Mazzone, Graciela Luján
author Di Mauro, Giuliana Constanza
author_facet Di Mauro, Giuliana Constanza
De Ambrosi, Bruno
Uchitel, Osvaldo Daniel
Mazzone, Graciela Luján
author_role author
author2 De Ambrosi, Bruno
Uchitel, Osvaldo Daniel
Mazzone, Graciela Luján
author2_role author
author
author
dc.subject.none.fl_str_mv AMYOTROPHIC LATERAL SCLEROSIS
AUTOIMMUNITY
MICROGLIAL ACTIVATION
NEURON LABELING
topic AMYOTROPHIC LATERAL SCLEROSIS
AUTOIMMUNITY
MICROGLIAL ACTIVATION
NEURON LABELING
purl_subject.fl_str_mv https://purl.org/becyt/ford/1.6
https://purl.org/becyt/ford/1
dc.description.none.fl_txt_mv Amyotrophic Lateral Sclerosis (ALS) is a degenerative disorder characterized by ongoing loss of motoneurons. The etiology of the sporadic ALS formit is thought to be related to immune-mediated motoneuron degeneration and death. The present study was designed to describe the effect of serumfactors derived from sporadic ALS patients on mouse spinal cord preparations in vitro. Sera from patients with sporadic ALS were collected and usedfor immunofluorescence analysis to investigate their effects on neuronal survival and microgliosis. Our experiments demonstrated that 5 h applicationof serum factors (derived from three ALS patients) labeled with human IgG secondary antibody were localized to ventral spinal neurons identified withthe NeuN marker. Moreover, a significant reduction in the number of ventral NeuN positive cells was observed with serum from a patient who sufferedfrom upper motor neuron signs as criteria for ALS diagnosis (20% less compared to sham, spinal cord preparations without serum). No change inmicroglia number was found after exposure to ALS sera, although in two cases a significant decrease in microglial branch length was observed (28-32%).Microglia morphology showed increased number of end points and branches with serum from the patient with upper motor neuron symptoms. Theseobservations were absent after control sera. Our data indicate that spinal cord cultures can be a useful model to further characterize the pathologicalprocesses of sporadic ALS and the immune mechanism as previously suggested in vivo. Future studies are needed to unveil the molecular mechanismsunderlying this preferential targeting of neurons and microglia by ALS serum.
Fil: Di Mauro, Giuliana Constanza. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Fisiología, Biología Molecular y Neurociencias. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Fisiología, Biología Molecular y Neurociencias; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigación en Biomedicina de Buenos Aires - Instituto Partner de la Sociedad Max Planck; Argentina
Fil: De Ambrosi, Bruno. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; Argentina
Fil: Uchitel, Osvaldo Daniel. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Fisiología, Biología Molecular y Neurociencias. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Fisiología, Biología Molecular y Neurociencias; Argentina. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; Argentina
Fil: Mazzone, Graciela Luján. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; Argentina. Universidad Austral. Facultad de Ciencias Biomédicas. Instituto de Investigaciones en Medicina Traslacional. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigaciones en Medicina Traslacional; Argentina
description Amyotrophic Lateral Sclerosis (ALS) is a degenerative disorder characterized by ongoing loss of motoneurons. The etiology of the sporadic ALS formit is thought to be related to immune-mediated motoneuron degeneration and death. The present study was designed to describe the effect of serumfactors derived from sporadic ALS patients on mouse spinal cord preparations in vitro. Sera from patients with sporadic ALS were collected and usedfor immunofluorescence analysis to investigate their effects on neuronal survival and microgliosis. Our experiments demonstrated that 5 h applicationof serum factors (derived from three ALS patients) labeled with human IgG secondary antibody were localized to ventral spinal neurons identified withthe NeuN marker. Moreover, a significant reduction in the number of ventral NeuN positive cells was observed with serum from a patient who sufferedfrom upper motor neuron signs as criteria for ALS diagnosis (20% less compared to sham, spinal cord preparations without serum). No change inmicroglia number was found after exposure to ALS sera, although in two cases a significant decrease in microglial branch length was observed (28-32%).Microglia morphology showed increased number of end points and branches with serum from the patient with upper motor neuron symptoms. Theseobservations were absent after control sera. Our data indicate that spinal cord cultures can be a useful model to further characterize the pathologicalprocesses of sporadic ALS and the immune mechanism as previously suggested in vivo. Future studies are needed to unveil the molecular mechanismsunderlying this preferential targeting of neurons and microglia by ALS serum.
publishDate 2021
dc.date.none.fl_str_mv 2021-07
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/185057
Di Mauro, Giuliana Constanza; De Ambrosi, Bruno; Uchitel, Osvaldo Daniel; Mazzone, Graciela Luján; Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina; Research Open; Journal of Neurology and Neurocritical Care; 4; 2; 7-2021; 1-5
2771-9065
CONICET Digital
CONICET
url http://hdl.handle.net/11336/185057
identifier_str_mv Di Mauro, Giuliana Constanza; De Ambrosi, Bruno; Uchitel, Osvaldo Daniel; Mazzone, Graciela Luján; Case Report of Neuron-Binding IgGs in ALS Patient Serum in Argentina; Research Open; Journal of Neurology and Neurocritical Care; 4; 2; 7-2021; 1-5
2771-9065
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://researchopenworld.com/case-report-of-neuron-binding-iggs-in-als-patient-serum-in-argentina/
info:eu-repo/semantics/altIdentifier/doi/10.31038/JNNC.2021423
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/pdf
dc.publisher.none.fl_str_mv Research Open
publisher.none.fl_str_mv Research Open
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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score 12.982451

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