Enhanced Collagen Deposition in the Duodenum of Patients with Hyaline Fibromatosis Syndrome and Protein Losing Enteropathy

Enhanced Collagen Deposition in the Duodenum of Patients with Hyaline Fibromatosis Syndrome and Protein Losing Enteropathy

Hyaline fibromatosis syndrome (HFS), resulting from <i>ANTXR2</i> mutations, is an ultra-rare disease that causes intestinal lymphangiectasia and protein-losing enteropathy (PLE). The mechanisms leading to the gastrointestinal phenotype in these patients are not well defined. We present...

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Bibliographic Details
Main Authors: Jorik M. van Rijn, Lael Werner, Yusuf Aydemir, Joey M.A. Spronck, Ben Pode-Shakked, Marliek van Hoesel, Elee Shimshoni, Sylvie Polak-Charcon, Liron Talmi, Makbule Eren, Batia Weiss, Roderick H.J. Houwen, Iris Barshack, Raz Somech, Edward E.S. Nieuwenhuis, Irit Sagi, Annick Raas-Rothschild, Sabine Middendorp, Dror S. Shouval
Format: Article
Language:English
Published: MDPI AG 2020-11-01
Series:International Journal of Molecular Sciences
Subjects:
protein losing enteropathy
intestinal lymphangiectasia
organoids
extracellular matrix
ANTXR2
CMG2
Online Access:https://www.mdpi.com/1422-0067/21/21/8200

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https://www.mdpi.com/1422-0067/21/21/8200

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