The role of tubular cells in the pathogenesis of Fabry nephropathy

Autores
Rozenfeld, Paula Adriana; Feriozzi, Sandro; Braun, Fabian
Año de publicación
2024
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
The pathophysiology of Fabry nephropathy (FN) is induced by galactosidase Adeficiency with a chronic exposure of glycolipids to every lineage of renalcells. Tissue damage is attributed to the activation of molecular pathways,resulting in tissue fibrosis and chronic kidney disease. Podocytes have beenthe primary focus in clinical pathophysiological research because of thestriking accumulation of large glycolipid deposits observable in histology. Yet,the tubular interstitium makes up a large portion of the whole organ, andtherefore, its role must be further considered in pathogenic processes. In thisreview, we would like to propose Fabry tubulopathy and its ensuing functionaleffects as the first pathological signs and contributing factors to thedevelopment of FN. We will summarize and discuss the current literatureregarding the role of tubular cells in Fabry kidney pathophysiology. Startingfrom clinical and histological evidence, we will highlight the data from animalmodels and cell cultures outlining the pathophysiological pathways associatedwith tubular interstitial injury causing renal fibrosis in Fabry nephropathy.
Fil: Rozenfeld, Paula Adriana. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Instituto de Estudios Inmunológicos y Fisiopatológicos. Universidad Nacional de La Plata. Facultad de Ciencias Exactas. Instituto de Estudios Inmunológicos y Fisiopatológicos; Argentina
Fil: Feriozzi, Sandro. Belcolle Hospital; Italia
Fil: Braun, Fabian. University Medical Center Hamburg-Eppendorf; Alemania
Materia
FABRY DISEASE
NEPHROPATHY
TUBULAR CELLS
FIBROSIS
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:11336/257298
Acceder
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network_name_str CONICET Digital (CONICET)
spelling The role of tubular cells in the pathogenesis of Fabry nephropathyRozenfeld, Paula AdrianaFeriozzi, SandroBraun, FabianFABRY DISEASENEPHROPATHYTUBULAR CELLSFIBROSIShttps://purl.org/becyt/ford/3.1https://purl.org/becyt/ford/3The pathophysiology of Fabry nephropathy (FN) is induced by galactosidase Adeficiency with a chronic exposure of glycolipids to every lineage of renalcells. Tissue damage is attributed to the activation of molecular pathways,resulting in tissue fibrosis and chronic kidney disease. Podocytes have beenthe primary focus in clinical pathophysiological research because of thestriking accumulation of large glycolipid deposits observable in histology. Yet,the tubular interstitium makes up a large portion of the whole organ, andtherefore, its role must be further considered in pathogenic processes. In thisreview, we would like to propose Fabry tubulopathy and its ensuing functionaleffects as the first pathological signs and contributing factors to thedevelopment of FN. We will summarize and discuss the current literatureregarding the role of tubular cells in Fabry kidney pathophysiology. Startingfrom clinical and histological evidence, we will highlight the data from animalmodels and cell cultures outlining the pathophysiological pathways associatedwith tubular interstitial injury causing renal fibrosis in Fabry nephropathy.Fil: Rozenfeld, Paula Adriana. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Instituto de Estudios Inmunológicos y Fisiopatológicos. Universidad Nacional de La Plata. Facultad de Ciencias Exactas. Instituto de Estudios Inmunológicos y Fisiopatológicos; ArgentinaFil: Feriozzi, Sandro. Belcolle Hospital; ItaliaFil: Braun, Fabian. University Medical Center Hamburg-Eppendorf; AlemaniaFrontiers Media2024-04info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/257298Rozenfeld, Paula Adriana; Feriozzi, Sandro; Braun, Fabian; The role of tubular cells in the pathogenesis of Fabry nephropathy; Frontiers Media; Frontiers in Cardiovascular Medicine; 11; 4-2024; 1-72297-055XCONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://www.frontiersin.org/articles/10.3389/fcvm.2024.1386042/fullinfo:eu-repo/semantics/altIdentifier/doi/10.3389/fcvm.2024.1386042info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-10T13:16:34Zoai:ri.conicet.gov.ar:11336/257298instacron: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:34982025-09-10 13:16:34.64CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv The role of tubular cells in the pathogenesis of Fabry nephropathy
title The role of tubular cells in the pathogenesis of Fabry nephropathy
spellingShingle The role of tubular cells in the pathogenesis of Fabry nephropathy
Rozenfeld, Paula Adriana
FABRY DISEASE
NEPHROPATHY
TUBULAR CELLS
FIBROSIS
title_short The role of tubular cells in the pathogenesis of Fabry nephropathy
title_full The role of tubular cells in the pathogenesis of Fabry nephropathy
title_fullStr The role of tubular cells in the pathogenesis of Fabry nephropathy
title_full_unstemmed The role of tubular cells in the pathogenesis of Fabry nephropathy
title_sort The role of tubular cells in the pathogenesis of Fabry nephropathy
dc.creator.none.fl_str_mv Rozenfeld, Paula Adriana
Feriozzi, Sandro
Braun, Fabian
author Rozenfeld, Paula Adriana
author_facet Rozenfeld, Paula Adriana
Feriozzi, Sandro
Braun, Fabian
author_role author
author2 Feriozzi, Sandro
Braun, Fabian
author2_role author
author
dc.subject.none.fl_str_mv FABRY DISEASE
NEPHROPATHY
TUBULAR CELLS
FIBROSIS
topic FABRY DISEASE
NEPHROPATHY
TUBULAR CELLS
FIBROSIS
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 pathophysiology of Fabry nephropathy (FN) is induced by galactosidase Adeficiency with a chronic exposure of glycolipids to every lineage of renalcells. Tissue damage is attributed to the activation of molecular pathways,resulting in tissue fibrosis and chronic kidney disease. Podocytes have beenthe primary focus in clinical pathophysiological research because of thestriking accumulation of large glycolipid deposits observable in histology. Yet,the tubular interstitium makes up a large portion of the whole organ, andtherefore, its role must be further considered in pathogenic processes. In thisreview, we would like to propose Fabry tubulopathy and its ensuing functionaleffects as the first pathological signs and contributing factors to thedevelopment of FN. We will summarize and discuss the current literatureregarding the role of tubular cells in Fabry kidney pathophysiology. Startingfrom clinical and histological evidence, we will highlight the data from animalmodels and cell cultures outlining the pathophysiological pathways associatedwith tubular interstitial injury causing renal fibrosis in Fabry nephropathy.
Fil: Rozenfeld, Paula Adriana. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Instituto de Estudios Inmunológicos y Fisiopatológicos. Universidad Nacional de La Plata. Facultad de Ciencias Exactas. Instituto de Estudios Inmunológicos y Fisiopatológicos; Argentina
Fil: Feriozzi, Sandro. Belcolle Hospital; Italia
Fil: Braun, Fabian. University Medical Center Hamburg-Eppendorf; Alemania
description The pathophysiology of Fabry nephropathy (FN) is induced by galactosidase Adeficiency with a chronic exposure of glycolipids to every lineage of renalcells. Tissue damage is attributed to the activation of molecular pathways,resulting in tissue fibrosis and chronic kidney disease. Podocytes have beenthe primary focus in clinical pathophysiological research because of thestriking accumulation of large glycolipid deposits observable in histology. Yet,the tubular interstitium makes up a large portion of the whole organ, andtherefore, its role must be further considered in pathogenic processes. In thisreview, we would like to propose Fabry tubulopathy and its ensuing functionaleffects as the first pathological signs and contributing factors to thedevelopment of FN. We will summarize and discuss the current literatureregarding the role of tubular cells in Fabry kidney pathophysiology. Startingfrom clinical and histological evidence, we will highlight the data from animalmodels and cell cultures outlining the pathophysiological pathways associatedwith tubular interstitial injury causing renal fibrosis in Fabry nephropathy.
publishDate 2024
dc.date.none.fl_str_mv 2024-04
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/11336/257298
Rozenfeld, Paula Adriana; Feriozzi, Sandro; Braun, Fabian; The role of tubular cells in the pathogenesis of Fabry nephropathy; Frontiers Media; Frontiers in Cardiovascular Medicine; 11; 4-2024; 1-7
2297-055X
CONICET Digital
CONICET
url http://hdl.handle.net/11336/257298
identifier_str_mv Rozenfeld, Paula Adriana; Feriozzi, Sandro; Braun, Fabian; The role of tubular cells in the pathogenesis of Fabry nephropathy; Frontiers Media; Frontiers in Cardiovascular Medicine; 11; 4-2024; 1-7
2297-055X
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://www.frontiersin.org/articles/10.3389/fcvm.2024.1386042/full
info:eu-repo/semantics/altIdentifier/doi/10.3389/fcvm.2024.1386042
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 Frontiers Media
publisher.none.fl_str_mv Frontiers Media
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 12.993085

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