The role of tubular cells in the pathogenesis of Fabry nephropathy

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...

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Autores principales:	Rozenfeld, Paula Adriana, Feriozzi, Sandro, Braun,Fabian
Formato:	Articulo
Lenguaje:	Inglés
Publicado:	2024
Materias:	Biología Fabry disease nephropathy tubular cells pathogenesis fibrosis
Acceso en línea:	http://sedici.unlp.edu.ar/handle/10915/167339
Aporte de:	SEDICI (UNLP) de Universidad Nacional de La Plata

id	I19-R120-10915-167339
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spelling	I19-R120-10915-1673392024-06-18T20:10:27Z http://sedici.unlp.edu.ar/handle/10915/167339 The role of tubular cells in the pathogenesis of Fabry nephropathy Rozenfeld, Paula Adriana Feriozzi, Sandro Braun,Fabian 2024 2024-06-18T16:02:39Z en Biología Fabry disease nephropathy tubular cells pathogenesis fibrosis 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 Articulo Articulo http://creativecommons.org/licenses/by/4.0/ Creative Commons Attribution 4.0 International (CC BY 4.0) application/pdf
institution	Universidad Nacional de La Plata
institution_str	I-19
repository_str	R-120
collection	SEDICI (UNLP)
language	Inglés
topic	Biología Fabry disease nephropathy tubular cells pathogenesis fibrosis
spellingShingle	Biología Fabry disease nephropathy tubular cells pathogenesis fibrosis Rozenfeld, Paula Adriana Feriozzi, Sandro Braun,Fabian The role of tubular cells in the pathogenesis of Fabry nephropathy
topic_facet	Biología Fabry disease nephropathy tubular cells pathogenesis fibrosis
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.
format	Articulo Articulo
author	Rozenfeld, Paula Adriana Feriozzi, Sandro Braun,Fabian
author_facet	Rozenfeld, Paula Adriana Feriozzi, Sandro Braun,Fabian
author_sort	Rozenfeld, Paula Adriana
title	The role of tubular cells in the pathogenesis of Fabry nephropathy
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	role of tubular cells in the pathogenesis of fabry nephropathy
publishDate	2024
url	http://sedici.unlp.edu.ar/handle/10915/167339
work_keys_str_mv	AT rozenfeldpaulaadriana theroleoftubularcellsinthepathogenesisoffabrynephropathy AT feriozzisandro theroleoftubularcellsinthepathogenesisoffabrynephropathy AT braunfabian theroleoftubularcellsinthepathogenesisoffabrynephropathy AT rozenfeldpaulaadriana roleoftubularcellsinthepathogenesisoffabrynephropathy AT feriozzisandro roleoftubularcellsinthepathogenesisoffabrynephropathy AT braunfabian roleoftubularcellsinthepathogenesisoffabrynephropathy
_version_	1807223485455400960

The role of tubular cells in the pathogenesis of Fabry nephropathy

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