Biliary Atresia – emerging diagnostic and therapy opportunities

Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In this review, we describe recent progress in the understanding of liver development with a focus on cholangiocyte differentiation and how use of technical platforms, including rodent, zebrafish and org...

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Autores principales:	Urban Lendahl, Vincent C.H. Lui, Patrick H.Y. Chung, Paul K.H. Tam
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Biliary Atresia Cholangiocyte Cholangiopathy Pathomechanisms Disease Models Therapy Medicine R Medicine (General) R5-920
Acceso en línea:	https://doaj.org/article/668915f7c8c546829052a8cd35c683c9
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spelling	oai:doaj.org-article:668915f7c8c546829052a8cd35c683c92021-11-14T04:34:19ZBiliary Atresia – emerging diagnostic and therapy opportunities2352-396410.1016/j.ebiom.2021.103689https://doaj.org/article/668915f7c8c546829052a8cd35c683c92021-12-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2352396421004837https://doaj.org/toc/2352-3964Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In this review, we describe recent progress in the understanding of liver development with a focus on cholangiocyte differentiation and how use of technical platforms, including rodent, zebrafish and organoid models, advances our understanding of Biliary Atresia. This is followed by a description of potential pathomechanisms, such as autoimmune responses, inflammation, disturbed apical-basal cell polarity, primary cilia dysfunction as well as beta-amyloid accumulation. Finally, we describe current and emerging diagnostic opportunities and recent translation breakthroughs for Biliary Atresia in the area of emerging therapy development, including immunomodulation and organoid-based systems for liver and bile duct repair.Urban LendahlVincent C.H. LuiPatrick H.Y. ChungPaul K.H. TamElsevierarticleBiliary AtresiaCholangiocyteCholangiopathyPathomechanismsDisease ModelsTherapyMedicineRMedicine (General)R5-920ENEBioMedicine, Vol 74, Iss , Pp 103689- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Biliary Atresia Cholangiocyte Cholangiopathy Pathomechanisms Disease Models Therapy Medicine R Medicine (General) R5-920
spellingShingle	Biliary Atresia Cholangiocyte Cholangiopathy Pathomechanisms Disease Models Therapy Medicine R Medicine (General) R5-920 Urban Lendahl Vincent C.H. Lui Patrick H.Y. Chung Paul K.H. Tam Biliary Atresia – emerging diagnostic and therapy opportunities
description	Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In this review, we describe recent progress in the understanding of liver development with a focus on cholangiocyte differentiation and how use of technical platforms, including rodent, zebrafish and organoid models, advances our understanding of Biliary Atresia. This is followed by a description of potential pathomechanisms, such as autoimmune responses, inflammation, disturbed apical-basal cell polarity, primary cilia dysfunction as well as beta-amyloid accumulation. Finally, we describe current and emerging diagnostic opportunities and recent translation breakthroughs for Biliary Atresia in the area of emerging therapy development, including immunomodulation and organoid-based systems for liver and bile duct repair.
format	article
author	Urban Lendahl Vincent C.H. Lui Patrick H.Y. Chung Paul K.H. Tam
author_facet	Urban Lendahl Vincent C.H. Lui Patrick H.Y. Chung Paul K.H. Tam
author_sort	Urban Lendahl
title	Biliary Atresia – emerging diagnostic and therapy opportunities
title_short	Biliary Atresia – emerging diagnostic and therapy opportunities
title_full	Biliary Atresia – emerging diagnostic and therapy opportunities
title_fullStr	Biliary Atresia – emerging diagnostic and therapy opportunities
title_full_unstemmed	Biliary Atresia – emerging diagnostic and therapy opportunities
title_sort	biliary atresia – emerging diagnostic and therapy opportunities
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/668915f7c8c546829052a8cd35c683c9
work_keys_str_mv	AT urbanlendahl biliaryatresiaemergingdiagnosticandtherapyopportunities AT vincentchlui biliaryatresiaemergingdiagnosticandtherapyopportunities AT patrickhychung biliaryatresiaemergingdiagnosticandtherapyopportunities AT paulkhtam biliaryatresiaemergingdiagnosticandtherapyopportunities
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Biliary Atresia – emerging diagnostic and therapy opportunities

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