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BRIEF RESEARCH REPORT article

Front. Cell Dev. Biol.
Sec. Stem Cell Research
Volume 13 - 2025 | doi: 10.3389/fcell.2025.1508714
This article is part of the Research Topic Advancements in Molecular and Cellular Mechanisms of Stem Cells in Tissue Development and Regeneration View all 5 articles

The Batten disease gene Cln3 is required for the activation of intestinal stem cell during regeneration via JAK/STAT signaling in Drosophila

Provisionally accepted
Haiyang Chen Haiyang Chen 1*Zihua Yu Zihua Yu 1,2Yan Jinhua Yan Jinhua 1Zhiming Liu Zhiming Liu 1Haiyan Wang Haiyan Wang 1Guan-Zheng Luo Guan-Zheng Luo 2
  • 1 West China Hospital, Sichuan University, Chengdu, China
  • 2 School of Life Sciences, Sun Yat-sen University, Guangzhou, Guangdong Province, China

The final, formatted version of the article will be published soon.

    CLN3 mutation causes Juvenile neuronal ceroid lipofuscinosis (JNCL, also known as Batten disease), an early onset neurodegenerative disorder. Patients who suffer from Batten disease often die at an early age. However, the mechanisms underlying how CLN3 loss develops Batten disease remain largely unclear. Here, using Drosophila midgut system, we demonstrate that Drosophila Cln3 has no effect on midgut homeostasis maintaince, including cellular component, intestinal stem cells (ISCs) proliferation and differentiation, but is necessary for ISC activation upon tissue damage. Cell type-specific Gal4 screening reveals that the failure of ISC activation during regeneration caused by Cln3 loss is ISC-autonomous. Through genetic analyses, we elucidate that Yu et al 2024 2 JAK/STAT signaling in ISCs is not activated with Cln3 depletion upon tissue damage, and functions downstream of Cln3. Our study provides a potential mechanism underlying the development of CLN3-mediated Batten disease at cellular level.

    Keywords: Drosophila, intestinal stem cell (ISC), Batten disease, CLN3, JAK - STAT signaling pathway

    Received: 09 Oct 2024; Accepted: 08 Jan 2025.

    Copyright: © 2025 Chen, Yu, Jinhua, Liu, Wang and Luo. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Haiyang Chen, West China Hospital, Sichuan University, Chengdu, China

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