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Biomolecules
Volume 16
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10.3390/biom16010009
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This is an early access version, the complete PDF, HTML, and XML versions will be available soon.
Article

Elevated Epithelial Splicing Regulatory Protein 1 Expression in Biliary Atresia Indicates Its Potential as a Molecular Marker

by
Giorgia Ammirata
1,†,
Victor Navarro-Tableros
1,2,†,
Marta Manco
1,
Ghania Zubair
3,
Luca Di Costanzo
1,
Luigi Chiusa
4,
Alice Ponte
5,
Michele Pinon
5,
Renato Romagnoli
6,
Ralf Weiskirchen
7,
Paola Cassoni
4,
Pier Luigi Calvo
5,
Ugo Ala
8,‡,
Fiorella Altruda
1,‡ and
Sharmila Fagoonee
1,9,*,‡
1
Molecular Biotechnology Center “Guido Tarone”, University of Turin, 10126 Turin, Italy
2
2i3T-Incubatore di Imprese, University of Turin, 10126 Turin, Italy
3
Department of Mathematics “Giuseppe Peano”, University of Turin, 10126 Turin, Italy
4
Pathology Unit, Department of Medical Sciences, University of Turin and AOU Città Della Salute e Della Scienza, 10126 Turin, Italy
5
Pediatric Gastroenterology Unit, Regina Margherita Children’s Hospital, AOU Città Della Salute e Della Scienza di Torino, 10126 Turin, Italy
6
Liver Transplant Center, Department of Surgical Sciences, University of Turin and AOU Città Della Salute e Della Scienza, 10126 Turin, Italy
7
Institute of Molecular Pathobiochemistry, Experimental Gene Therapy and Clinical Chemistry (IFMPEGKC), RWTH University Hospital Aachen, D-52074 Aachen, Germany
8
Department of Veterinary Sciences, University of Turin, Grugliasco, 10095 Turin, Italy
9
Institute of Biostructure and Bioimaging (CNR), 10126 Turin, Italy
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
‡
These authors supervised equally to this work.
Biomolecules 2026, 16(1), 9; https://doi.org/10.3390/biom16010009
Submission received: 29 October 2025 / Revised: 5 December 2025 / Accepted: 15 December 2025 / Published: 19 December 2025
(This article belongs to the Special Issue Molecular, Cellular, and Blood Biomarkers in Diagnosis, Prognosis, Monitoring, and Treatment: 2nd Edition)
Versions Notes

Abstract

Cholangiopathies encompass a wide range of chronic liver diseases that target biliary epithelial cells, leading to significant morbidity and mortality due to their progressive nature, limited treatment options, and complex clinical management. Currently, clinically validated biomarkers capable of distinguishing obstructive cholangiopathies, such as biliary atresia (BA), from other cholangiopathies are lacking, hindering timely intervention. RNA-binding proteins (RBPs) have been increasingly linked to human diseases but their roles in cholangiopathies remain underexplored. We assessed the expression of the RBP epithelial splicing regulatory protein 1 (ESRP1) in murine models of cholangiopathies and in the human system. Our findings demonstrate that ESRP1 is highly and specifically expressed in cholestatic liver injury models, including bile duct-ligated, diethoxycarboncyl-1,4-dihydrocollidine-treated, and Mdr2−/− mice when compared with other liver injury models. Importantly, ESRP1 is markedly elevated in the livers of patients with BA and cystic fibrosis-related liver disease, localizing to cholangiocytes and peri-biliary hepatic cells, but is minimal in primary sclerosing cholangitis and primary biliary cholangitis. Moreover, patient-derived BA organoids and biliatresone-treated healthy organoids also display ESRP1 expression. Bioinformatics analysis further implicates ESRP1 in key cholangiopathy-associated pathways, warranting deeper mechanistic investigation. Thus, ESRP1 holds potential as a molecular marker for obstructive cholangiopathies, warranting further mechanistic studies.
Keywords: ESRP1; hepatobiliary system; cholangiocyte organoids; bioinformatics; cholestasis marker; Biliary Atresia; CFLD; PSC; PBC; mouse models ESRP1; hepatobiliary system; cholangiocyte organoids; bioinformatics; cholestasis marker; Biliary Atresia; CFLD; PSC; PBC; mouse models

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MDPI and ACS Style

Ammirata, G.; Navarro-Tableros, V.; Manco, M.; Zubair, G.; Di Costanzo, L.; Chiusa, L.; Ponte, A.; Pinon, M.; Romagnoli, R.; Weiskirchen, R.; et al. Elevated Epithelial Splicing Regulatory Protein 1 Expression in Biliary Atresia Indicates Its Potential as a Molecular Marker. Biomolecules 2026, 16, 9. https://doi.org/10.3390/biom16010009

AMA Style

Ammirata G, Navarro-Tableros V, Manco M, Zubair G, Di Costanzo L, Chiusa L, Ponte A, Pinon M, Romagnoli R, Weiskirchen R, et al. Elevated Epithelial Splicing Regulatory Protein 1 Expression in Biliary Atresia Indicates Its Potential as a Molecular Marker. Biomolecules. 2026; 16(1):9. https://doi.org/10.3390/biom16010009

Chicago/Turabian Style

Ammirata, Giorgia, Victor Navarro-Tableros, Marta Manco, Ghania Zubair, Luca Di Costanzo, Luigi Chiusa, Alice Ponte, Michele Pinon, Renato Romagnoli, Ralf Weiskirchen, and et al. 2026. "Elevated Epithelial Splicing Regulatory Protein 1 Expression in Biliary Atresia Indicates Its Potential as a Molecular Marker" Biomolecules 16, no. 1: 9. https://doi.org/10.3390/biom16010009

APA Style

Ammirata, G., Navarro-Tableros, V., Manco, M., Zubair, G., Di Costanzo, L., Chiusa, L., Ponte, A., Pinon, M., Romagnoli, R., Weiskirchen, R., Cassoni, P., Calvo, P. L., Ala, U., Altruda, F., & Fagoonee, S. (2026). Elevated Epithelial Splicing Regulatory Protein 1 Expression in Biliary Atresia Indicates Its Potential as a Molecular Marker. Biomolecules, 16(1), 9. https://doi.org/10.3390/biom16010009

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