Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal Microscopy

Several clinical trials are working on drug development for Duchenne and Becker muscular dystrophy (DMD and BMD) treatment, and, since the expected increase in dystrophin is relatively subtle, high-sensitivity quantification methods are necessary. There is also a need to quantify dystrophin to reach...

Descripción completa

Detalles Bibliográficos
Autores: Codina A, Roldán M, Natera-de Benito D, Ortez C, Planas R, Matalonga L, Cuadras D, Carrera L, Exposito J, Marquez J, Jimenez-Mallebrera C, M Porta J, Nascimento A, Jou C
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2023
País:España
Institución:Fundació Sant Joan de Déu
Repositorio:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:fsjd.fundanetsuite.com:p23202
Acceso en línea:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=23202
Access Level:acceso abierto
Palabra clave:dystrophin
Duchenne muscular dystrophy
Becker muscular dystrophy
confocal microscopy
fluorescence quantification
spectral imaging
id ES_fa5c6f8ec5edfd4fb3a1b41d08a5d114
oai_identifier_str oai:fsjd.fundanetsuite.com:p23202
network_acronym_str ES
network_name_str España
repository_id_str
spelling Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal MicroscopyCodina ARoldán MNatera-de Benito DOrtez CPlanas RMatalonga LCuadras DCarrera LExposito JMarquez JJimenez-Mallebrera CM Porta JNascimento AJou CdystrophinDuchenne muscular dystrophyBecker muscular dystrophyconfocal microscopyfluorescence quantificationspectral imagingSeveral clinical trials are working on drug development for Duchenne and Becker muscular dystrophy (DMD and BMD) treatment, and, since the expected increase in dystrophin is relatively subtle, high-sensitivity quantification methods are necessary. There is also a need to quantify dystrophin to reach a definitive diagnosis in individuals with mild BMD, and in female carriers. We developed a method for the quantification of dystrophin in DMD and BMD patients using spectral confocal microscopy. It offers the possibility to capture the whole emission spectrum for any antibody, ensuring the selection of the emission peak and allowing the detection of fluorescent emissions of very low intensities. Fluorescence was evaluated first on manually selected regions of interest (ROIs), proving the usefulness of the methodology. Later, ROI selection was automated to make it operator-independent. The proposed methodology correctly classified patients according to their diagnosis, detected even minimal traces of dystrophin, and the results obtained automatically were statistically comparable to the manual ones. Thus, spectral imaging could be implemented to measure dystrophin expression and it could pave the way for detailed analysis of how its expression relates to the clinical course. Studies could be further expanded to better understand the expression of dystrophin-associated protein complexes (DAPCs).MDPI2023info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=23202INTERNATIONAL JOURNAL OF MOLECULAR SCIENCESISSN: 16616596ISSNe: 14220067reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déuinstname:Fundació Sant Joan de DéuInglésinfo:eu-repo/semantics/openAccessoai:fsjd.fundanetsuite.com:p232022026-05-27T12:37:41Z
dc.title.none.fl_str_mv Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal Microscopy
title Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal Microscopy
spellingShingle Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal Microscopy
Codina A
dystrophin
Duchenne muscular dystrophy
Becker muscular dystrophy
confocal microscopy
fluorescence quantification
spectral imaging
title_short Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal Microscopy
title_full Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal Microscopy
title_fullStr Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal Microscopy
title_full_unstemmed Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal Microscopy
title_sort Innovative Computerized Dystrophin Quantification Method Based on Spectral Confocal Microscopy
dc.creator.none.fl_str_mv Codina A
Roldán M
Natera-de Benito D
Ortez C
Planas R
Matalonga L
Cuadras D
Carrera L
Exposito J
Marquez J
Jimenez-Mallebrera C
M Porta J
Nascimento A
Jou C
author Codina A
author_facet Codina A
Roldán M
Natera-de Benito D
Ortez C
Planas R
Matalonga L
Cuadras D
Carrera L
Exposito J
Marquez J
Jimenez-Mallebrera C
M Porta J
Nascimento A
Jou C
author_role author
author2 Roldán M
Natera-de Benito D
Ortez C
Planas R
Matalonga L
Cuadras D
Carrera L
Exposito J
Marquez J
Jimenez-Mallebrera C
M Porta J
Nascimento A
Jou C
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv dystrophin
Duchenne muscular dystrophy
Becker muscular dystrophy
confocal microscopy
fluorescence quantification
spectral imaging
topic dystrophin
Duchenne muscular dystrophy
Becker muscular dystrophy
confocal microscopy
fluorescence quantification
spectral imaging
description Several clinical trials are working on drug development for Duchenne and Becker muscular dystrophy (DMD and BMD) treatment, and, since the expected increase in dystrophin is relatively subtle, high-sensitivity quantification methods are necessary. There is also a need to quantify dystrophin to reach a definitive diagnosis in individuals with mild BMD, and in female carriers. We developed a method for the quantification of dystrophin in DMD and BMD patients using spectral confocal microscopy. It offers the possibility to capture the whole emission spectrum for any antibody, ensuring the selection of the emission peak and allowing the detection of fluorescent emissions of very low intensities. Fluorescence was evaluated first on manually selected regions of interest (ROIs), proving the usefulness of the methodology. Later, ROI selection was automated to make it operator-independent. The proposed methodology correctly classified patients according to their diagnosis, detected even minimal traces of dystrophin, and the results obtained automatically were statistically comparable to the manual ones. Thus, spectral imaging could be implemented to measure dystrophin expression and it could pave the way for detailed analysis of how its expression relates to the clinical course. Studies could be further expanded to better understand the expression of dystrophin-associated protein complexes (DAPCs).
publishDate 2023
dc.date.none.fl_str_mv 2023
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://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=23202
url https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=23202
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv MDPI
publisher.none.fl_str_mv MDPI
dc.source.none.fl_str_mv INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
ISSN: 16616596
ISSNe: 14220067
reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
instname:Fundació Sant Joan de Déu
instname_str Fundació Sant Joan de Déu
reponame_str r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
collection r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869425204094042112
score 15,812429

Similares en LA Referencia