Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's disease

In neurodegenerative diseases, neuronal dysfunction and degeneration start many years before the emergence of clinical symptoms. An important goal to advance in their knowledge and treatment is the identification of peripherical biomarkers to predict the onset and progression and to test the efficac...

Descripción completa

Detalles Bibliográficos
Autores: García Forn, Marta, Castany Pladevall, Carla, Golbano, Arantxa, Pérez Pérez, Jesús, Brito, Verónica, Kilisevsky, Jaime, Pérez Navarro, Ester
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2023
País:España
Institución:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:2445/218920
Acceso en línea:https://hdl.handle.net/2445/218920
Access Level:acceso abierto
Palabra clave:Medicina experimental
Corea de Huntington
Marcadors bioquímics
Experimental medicine
Huntington's chorea
Biochemical markers
id ES_f0427bae3c650d5d2519aec6f2abebe1
oai_identifier_str oai:recercat.cat:2445/218920
network_acronym_str ES
network_name_str España
repository_id_str
spelling Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's diseaseGarcía Forn, MartaCastany Pladevall, CarlaGolbano, ArantxaPérez Pérez, JesúsBrito, VerónicaKilisevsky, JaimePérez Navarro, EsterMedicina experimentalCorea de HuntingtonMarcadors bioquímicsExperimental medicineHuntington's choreaBiochemical markersIn neurodegenerative diseases, neuronal dysfunction and degeneration start many years before the emergence of clinical symptoms. An important goal to advance in their knowledge and treatment is the identification of peripherical biomarkers to predict the onset and progression and to test the efficacy of therapies. 1 We have previously shown that alterations in lamin B1, a member of the lamin family of proteins that are crucial for nuclear functionality, 2 are involved in the pathophysiology of Huntington's disease (HD). Specifically, lamin B1 levels are increased in the R6/1 HD mouse model at the onset of motor symptoms in striatal medium‐sized spiny and CA1 hippocampal neurons nuclei in correlation with nuclear dysfunction. 3 Therefore, we asked if increased lamin B1 levels and/or alterations in nuclear morphology could be also occurring in more accessible cells, namely fibroblasts and blood cells, and serve as biomarkers of the disease progression and/or treatment efficacy.John Wiley & Sons2025202520232025info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion6 p.application/pdfhttps://hdl.handle.net/2445/218920Articles publicats en revistes (Biomedicina)reponame:Recercat. Dipósit de la Recerca de Catalunyainstname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)InglésReproducció del document publicat a: https://doi.org/10.1002/ctm2.1154Clinical and Translational Medicine, 2023, num.2https://doi.org/10.1002/ctm2.1154cc-by (c) Garcia-Forn M et al., 2023http://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:recercat.cat:2445/2189202026-05-29T05:05:01Z
dc.title.none.fl_str_mv Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's disease
title Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's disease
spellingShingle Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's disease
García Forn, Marta
Medicina experimental
Corea de Huntington
Marcadors bioquímics
Experimental medicine
Huntington's chorea
Biochemical markers
title_short Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's disease
title_full Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's disease
title_fullStr Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's disease
title_full_unstemmed Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's disease
title_sort Lamin B1 and nuclear morphology in peripheral cells as new potential biomarkers to follow treatment response in Huntington's disease
dc.creator.none.fl_str_mv García Forn, Marta
Castany Pladevall, Carla
Golbano, Arantxa
Pérez Pérez, Jesús
Brito, Verónica
Kilisevsky, Jaime
Pérez Navarro, Ester
author García Forn, Marta
author_facet García Forn, Marta
Castany Pladevall, Carla
Golbano, Arantxa
Pérez Pérez, Jesús
Brito, Verónica
Kilisevsky, Jaime
Pérez Navarro, Ester
author_role author
author2 Castany Pladevall, Carla
Golbano, Arantxa
Pérez Pérez, Jesús
Brito, Verónica
Kilisevsky, Jaime
Pérez Navarro, Ester
author2_role author
author
author
author
author
author
dc.subject.none.fl_str_mv Medicina experimental
Corea de Huntington
Marcadors bioquímics
Experimental medicine
Huntington's chorea
Biochemical markers
topic Medicina experimental
Corea de Huntington
Marcadors bioquímics
Experimental medicine
Huntington's chorea
Biochemical markers
description In neurodegenerative diseases, neuronal dysfunction and degeneration start many years before the emergence of clinical symptoms. An important goal to advance in their knowledge and treatment is the identification of peripherical biomarkers to predict the onset and progression and to test the efficacy of therapies. 1 We have previously shown that alterations in lamin B1, a member of the lamin family of proteins that are crucial for nuclear functionality, 2 are involved in the pathophysiology of Huntington's disease (HD). Specifically, lamin B1 levels are increased in the R6/1 HD mouse model at the onset of motor symptoms in striatal medium‐sized spiny and CA1 hippocampal neurons nuclei in correlation with nuclear dysfunction. 3 Therefore, we asked if increased lamin B1 levels and/or alterations in nuclear morphology could be also occurring in more accessible cells, namely fibroblasts and blood cells, and serve as biomarkers of the disease progression and/or treatment efficacy.
publishDate 2023
dc.date.none.fl_str_mv 2023
2025
2025
2025
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/2445/218920
url https://hdl.handle.net/2445/218920
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Reproducció del document publicat a: https://doi.org/10.1002/ctm2.1154
Clinical and Translational Medicine, 2023, num.2
https://doi.org/10.1002/ctm2.1154
dc.rights.none.fl_str_mv cc-by (c) Garcia-Forn M et al., 2023
http://creativecommons.org/licenses/by/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv cc-by (c) Garcia-Forn M et al., 2023
http://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 6 p.
application/pdf
dc.publisher.none.fl_str_mv John Wiley & Sons
publisher.none.fl_str_mv John Wiley & Sons
dc.source.none.fl_str_mv Articles publicats en revistes (Biomedicina)
reponame:Recercat. Dipósit de la Recerca de Catalunya
instname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
instname_str Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
reponame_str Recercat. Dipósit de la Recerca de Catalunya
collection Recercat. Dipósit de la Recerca de Catalunya
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869423940527456256
score 15,223283

Similares en LA Referencia