MBNL1 Promotes Intestinal Fibrosis via RAS-MAPK Pathway-Mediated Fibroblast Activation and Proliferation

Background: Intestinal fibrosis is a severe complication of Crohn’s disease (CD) with no effective therapies currently available. Muscleblind-like protein 1 (MBNL1) is an RNA-binding protein that has been implicated in fibrosis across multiple organs, but its role in CD-associated intestinal fibrosis remains unexplored. This study aims to investigate the expression, functional role, and underlying mechanism of MBNL1 in intestinal fibrosis. Methods: MBNL1 expression was examined in a TNBS-induced mouse model and in stenotic intestinal tissues from CD patients. In vitro, human colonic fibroblasts (CCD-18Co) were stimulated with transforming growth factor-β1 (TGF-β1) to model fibrosis. MBNL1 was knocked down or overexpressed to assess its effects on fibroblast activation, proliferation (5-ethynyl-2′-deoxyuridine, EdU; Cell Counting Kit-8, CCK-8), and apoptosis (flow cytometry). Potential downstream pathways were predicted using BioGRID and DAVID analyses and validated by Western blot. A rescue experiment with the RAS activator ML-097 was performed to confirm pathway dependency. Results: MBNL1 expression was significantly upregulated in fibrotic tissues from both the mouse model and CD patients, as well as in TGF-β1-stimulated CCD-18Co. MBNL1 knockdown suppressed TGF-β1-induced fibroblast activation and proliferation while promoting apoptosis, whereas MBNL1 overexpression had the opposite effect. Mechanistically, MBNL1 positively regulated the RAS-MAPK signaling pathway. Reactivation of this pathway with ML-097 reversed the inhibitory effects of MBNL1 knockdown on fibroblast activation and proliferation. Conclusions: MBNL1 promotes colonic fibroblast activation and proliferation by activating the RAS-MAPK signaling pathway, establishing it as a potential therapeutic target for intestinal fibrosis in Crohn’s disease.

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Publication Details

Journal
Biomedicines
Published
2026-05-27
DOI
https://doi.org/10.3390/biomedicines14061207
Primary Topic
Muscle Physiology and Disorders
Type
article
Field-Weighted Citation Impact
0.00

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article

MBNL1 Promotes Intestinal Fibrosis via RAS-MAPK Pathway-Mediated Fibroblast Activation and Proliferation

Ruijian Zhang, Li Zhang, Xi Zhang, Tianqi Liu et al.
Biomedicines
Muscle Physiology and Disorders
article

MBNL1 Promotes Intestinal Fibrosis via RAS-MAPK Pathway-Mediated Fibroblast Activation and Proliferation

Ruijian Zhang, Li Zhang, Xi Zhang, Tianqi Liu, Na Yu, Yi-Xiang Wang, Zhepeng Luo
article en

Abstract

Background: Intestinal fibrosis is a severe complication of Crohn’s disease (CD) with no effective therapies currently available. Muscleblind-like protein 1 (MBNL1) is an RNA-binding protein that has been implicated in fibrosis across multiple organs, but its role in CD-associated intestinal fibrosis remains unexplored. This study aims to investigate the expression, functional role, and underlying mechanism of MBNL1 in intestinal fibrosis. Methods: MBNL1 expression was examined in a TNBS-induced mouse model and in stenotic intestinal tissues from CD patients. In vitro, human colonic fibroblasts (CCD-18Co) were stimulated with transforming growth factor-β1 (TGF-β1) to model fibrosis. MBNL1 was knocked down or overexpressed to assess its effects on fibroblast activation, proliferation (5-ethynyl-2′-deoxyuridine, EdU; Cell Counting Kit-8, CCK-8), and apoptosis (flow cytometry). Potential downstream pathways were predicted using BioGRID and DAVID analyses and validated by Western blot. A rescue experiment with the RAS activator ML-097 was performed to confirm pathway dependency. Results: MBNL1 expression was significantly upregulated in fibrotic tissues from both the mouse model and CD patients, as well as in TGF-β1-stimulated CCD-18Co. MBNL1 knockdown suppressed TGF-β1-induced fibroblast activation and proliferation while promoting apoptosis, whereas MBNL1 overexpression had the opposite effect. Mechanistically, MBNL1 positively regulated the RAS-MAPK signaling pathway. Reactivation of this pathway with ML-097 reversed the inhibitory effects of MBNL1 knockdown on fibroblast activation and proliferation. Conclusions: MBNL1 promotes colonic fibroblast activation and proliferation by activating the RAS-MAPK signaling pathway, establishing it as a potential therapeutic target for intestinal fibrosis in Crohn’s disease.

BiomedicinesVol. 14(6)
First Hospital of China Medical University (CN), China Medical University (CN)
National Natural Science Foundation of China
Good health and well-being
Openalex Percentile: Top 9%
Muscle Physiology and Disorders
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