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 A deficiency with a chronic exposure of glycolipids to every lineage of renal cells. Tissue damage is attributed to the activation of molecular pathways, resulting in tissue fibrosis and chronic kidney disease. Podocytes have been the primary focus in clinical pathophysiological research because of the striking accumulation of large glycolipid deposits observable in histology. Yet, the tubular interstitium makes up a large portion of the whole organ, and therefore, its role must be further considered in pathogenic processes. In this review, we would like to propose Fabry tubulopathy and its ensuing functional effects as the first pathological signs and contributing factors to the development of FN. We will summarize and discuss the current literature regarding the role of tubular cells in Fabry kidney pathophysiology. Starting from clinical and histological evidence, we will highlight the data from animal models and cell cultures outlining the pathophysiological pathways associated with tubular interstitial injury causing renal fibrosis in Fabry nephropathy.
Instituto de Estudios Inmunológicos y Fisiopatológicos
Materia
Biología
Fabry disease
nephropathy
tubular cells
pathogenesis
fibrosis
Nivel de accesibilidad
acceso abierto
Condiciones de uso
http://creativecommons.org/licenses/by/4.0/
Repositorio
SEDICI (UNLP)
Institución
Universidad Nacional de La Plata
OAI Identificador
oai:sedici.unlp.edu.ar:10915/167339
Acceder
id SEDICI_c728083aa2f6f938728244e31329a800
oai_identifier_str oai:sedici.unlp.edu.ar:10915/167339
network_acronym_str SEDICI
repository_id_str 1329
network_name_str SEDICI (UNLP)
spelling The role of tubular cells in the pathogenesis of Fabry nephropathyRozenfeld, Paula AdrianaFeriozzi, SandroBraun,FabianBiologíaFabry diseasenephropathytubular cellspathogenesisfibrosisThe pathophysiology of Fabry nephropathy (FN) is induced by galactosidase A deficiency with a chronic exposure of glycolipids to every lineage of renal cells. Tissue damage is attributed to the activation of molecular pathways, resulting in tissue fibrosis and chronic kidney disease. Podocytes have been the primary focus in clinical pathophysiological research because of the striking accumulation of large glycolipid deposits observable in histology. Yet, the tubular interstitium makes up a large portion of the whole organ, and therefore, its role must be further considered in pathogenic processes. In this review, we would like to propose Fabry tubulopathy and its ensuing functional effects as the first pathological signs and contributing factors to the development of FN. We will summarize and discuss the current literature regarding the role of tubular cells in Fabry kidney pathophysiology. Starting from clinical and histological evidence, we will highlight the data from animal models and cell cultures outlining the pathophysiological pathways associated with tubular interstitial injury causing renal fibrosis in Fabry nephropathy.Instituto de Estudios Inmunológicos y Fisiopatológicos2024info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionArticulohttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfhttp://sedici.unlp.edu.ar/handle/10915/167339enginfo:eu-repo/semantics/altIdentifier/issn/2297-055Xinfo:eu-repo/semantics/altIdentifier/doi/10.3389/fcvm.2024.1386042info:eu-repo/semantics/openAccesshttp://creativecommons.org/licenses/by/4.0/Creative Commons Attribution 4.0 International (CC BY 4.0)reponame:SEDICI (UNLP)instname:Universidad Nacional de La Platainstacron:UNLP2025-10-15T11:36:21Zoai:sedici.unlp.edu.ar:10915/167339Institucionalhttp://sedici.unlp.edu.ar/Universidad públicaNo correspondehttp://sedici.unlp.edu.ar/oai/snrdalira@sedici.unlp.edu.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:13292025-10-15 11:36:21.5SEDICI (UNLP) - Universidad Nacional de La Platafalse
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
Biología
Fabry disease
nephropathy
tubular cells
pathogenesis
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 Biología
Fabry disease
nephropathy
tubular cells
pathogenesis
fibrosis
topic Biología
Fabry disease
nephropathy
tubular cells
pathogenesis
fibrosis
dc.description.none.fl_txt_mv The pathophysiology of Fabry nephropathy (FN) is induced by galactosidase A deficiency with a chronic exposure of glycolipids to every lineage of renal cells. Tissue damage is attributed to the activation of molecular pathways, resulting in tissue fibrosis and chronic kidney disease. Podocytes have been the primary focus in clinical pathophysiological research because of the striking accumulation of large glycolipid deposits observable in histology. Yet, the tubular interstitium makes up a large portion of the whole organ, and therefore, its role must be further considered in pathogenic processes. In this review, we would like to propose Fabry tubulopathy and its ensuing functional effects as the first pathological signs and contributing factors to the development of FN. We will summarize and discuss the current literature regarding the role of tubular cells in Fabry kidney pathophysiology. Starting from clinical and histological evidence, we will highlight the data from animal models and cell cultures outlining the pathophysiological pathways associated with tubular interstitial injury causing renal fibrosis in Fabry nephropathy.
Instituto de Estudios Inmunológicos y Fisiopatológicos
description The pathophysiology of Fabry nephropathy (FN) is induced by galactosidase A deficiency with a chronic exposure of glycolipids to every lineage of renal cells. Tissue damage is attributed to the activation of molecular pathways, resulting in tissue fibrosis and chronic kidney disease. Podocytes have been the primary focus in clinical pathophysiological research because of the striking accumulation of large glycolipid deposits observable in histology. Yet, the tubular interstitium makes up a large portion of the whole organ, and therefore, its role must be further considered in pathogenic processes. In this review, we would like to propose Fabry tubulopathy and its ensuing functional effects as the first pathological signs and contributing factors to the development of FN. We will summarize and discuss the current literature regarding the role of tubular cells in Fabry kidney pathophysiology. Starting from clinical and histological evidence, we will highlight the data from animal models and cell cultures outlining the pathophysiological pathways associated with tubular interstitial injury causing renal fibrosis in Fabry nephropathy.
publishDate 2024
dc.date.none.fl_str_mv 2024
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
Articulo
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://sedici.unlp.edu.ar/handle/10915/167339
url http://sedici.unlp.edu.ar/handle/10915/167339
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/issn/2297-055X
info:eu-repo/semantics/altIdentifier/doi/10.3389/fcvm.2024.1386042
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
http://creativecommons.org/licenses/by/4.0/
Creative Commons Attribution 4.0 International (CC BY 4.0)
eu_rights_str_mv openAccess
rights_invalid_str_mv http://creativecommons.org/licenses/by/4.0/
Creative Commons Attribution 4.0 International (CC BY 4.0)
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:SEDICI (UNLP)
instname:Universidad Nacional de La Plata
instacron:UNLP
reponame_str SEDICI (UNLP)
collection SEDICI (UNLP)
instname_str Universidad Nacional de La Plata
instacron_str UNLP
institution UNLP
repository.name.fl_str_mv SEDICI (UNLP) - Universidad Nacional de La Plata
repository.mail.fl_str_mv alira@sedici.unlp.edu.ar
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