Cystic fibrosis-related liver disease: clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

J Dana, D Debray, A Beaufrère, S Hillaire, M Fabre… - Journal of …, 2022 - Elsevier
Cystic fibrosis (CF) is the most common autosomal recessive disease in the Caucasian
population. Cystic fibrosis-related liver disease (CFLD) is defined as the pathogenesis …

[HTML][HTML] Animal models of biliary injury and altered bile acid metabolism

V Mariotti, M Strazzabosco, L Fabris… - Biochimica et Biophysica …, 2018 - Elsevier
In the last 25 years, a number of animal models, mainly rodents, have been generated with
the goal to mimic cholestatic liver injuries and, thus, to provide in vivo tools to investigate the …

Monocyte-derived alveolar macrophages drive lung fibrosis and persist in the lung over the life span

AV Misharin, L Morales-Nebreda… - Journal of Experimental …, 2017 - rupress.org
Little is known about the relative importance of monocyte and tissue-resident macrophages
in the development of lung fibrosis. We show that specific genetic deletion of monocyte …

Cystic fibrosis, CFTR, and colorectal cancer

P Scott, K Anderson, M Singhania… - International journal of …, 2020 - mdpi.com
Cystic fibrosis (CF), caused by biallelic inactivating mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene, has recently been categorized as a …

Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases

L Fabris, R Fiorotto, C Spirli, M Cadamuro… - Nature Reviews …, 2019 - nature.com
Bile duct epithelial cells, also known as cholangiocytes, regulate the composition of bile and
its flow. Acquired, congenital and genetic dysfunctions in these cells give rise to a set of …

[PDF][PDF] High mobility group protein B1 controls liver cancer initiation through yes‐associated protein‐dependent aerobic glycolysis

R Chen, S Zhu, XG Fan, H Wang, MT Lotze… - …, 2018 - Wiley Online Library
Emerging studies have suggested that the Hippo pathway is involved in the tumorigenesis of
hepatocellular carcinoma (HCC). However, the key regulator of the Hippo pathway in liver …

[HTML][HTML] Role of inflammation and proinflammatory cytokines in cholangiocyte pathophysiology

C Pinto, DM Giordano, L Maroni, M Marzioni - Biochimica et Biophysica …, 2018 - Elsevier
Cholangiocytes, the epithelial cells lining the bile ducts, are an important subset of liver
cells. They are involved in the modification of bile volume and composition, and respond to …

[HTML][HTML] Role of ion channels in gastrointestinal cancer

KJ Anderson, RT Cormier, PM Scott - World Journal of …, 2019 - ncbi.nlm.nih.gov
In their seminal papers Hanahan and Weinberg described oncogenic processes a normal
cell undergoes to be transformed into a cancer cell. The functions of ion channels in the …

Indole-3-carboxaldehyde restores gut mucosal integrity and protects from liver fibrosis in murine sclerosing cholangitis

F D'Onofrio, G Renga, M Puccetti, M Pariano… - Cells, 2021 - mdpi.com
Primary sclerosing cholangitis (PSC) is a long-term liver disease characterized by a
progressive course of cholestasis with liver inflammation and fibrosis. Intestinal barrier …

[PDF][PDF] B lymphocytes limit senescence‐driven fibrosis resolution and favor hepatocarcinogenesis in mouse liver injury

F Faggioli, E Palagano, L Di Tommaso… - …, 2018 - Wiley Online Library
Hepatocellular carcinoma (HCC) is a frequent neoplasia and a leading cause of
inflammation‐related cancer mortality. Despite that most HCCs arise from persistent …