Adaptive mechanisms in pancreatic islets counteract mitochondrial dysfunction in Barth syndrome
Our findings demonstrate that while Taz and cardiolipin remodelling are essential for beta cell physiology, systemic and islet-specific compensatory mechanisms preserve insulin secretion in vivo in Taz-KD mice, alongside increased glucagon secretion. These adaptations probably contribute to the altered metabolic phenotype observed in Barth syndrome and highlight a potential role for hormones and circulating factors such as FGF-21 in maintaining islet function and glucose homeostasis.
Authors
- Emmanuel Ampofo
- Patrick Gilon (ORCID: https://orcid.org/0000-0002-5885-8347)
- Karina von der Malsburg (ORCID: https://orcid.org/0009-0008-6596-3277)
- Letícia Prates Roma (ORCID: https://orcid.org/0000-0001-9527-0529)
- Andressa G. Amaral (ORCID: https://orcid.org/0000-0002-1261-2982)
- Marcel A. Lauterbach (ORCID: https://orcid.org/0009-0008-8145-6873)
- Edoardo Bertero (ORCID: https://orcid.org/0000-0002-3175-8151)
- Markus Hoth (ORCID: https://orcid.org/0000-0001-7080-4643)
- Jan Dudek (ORCID: https://orcid.org/0000-0002-5672-7458)
- Takahiro Higuchi (ORCID: https://orcid.org/0000-0001-9711-7835)
- Christoph Maack (ORCID: https://orcid.org/0000-0003-3694-4559)
- Monika Bozem (ORCID: https://orcid.org/0000-0002-1419-3099)
- Christopher Carlein (ORCID: https://orcid.org/0000-0001-5100-8932)
- Bruce Morgan (ORCID: https://orcid.org/0000-0001-9393-1071)
- Ahmadali Lotfinia
- Magalie A. Ravier (ORCID: https://orcid.org/0000-0001-6607-6559)
- Markus D. A. Hoffmann (ORCID: https://orcid.org/0000-0001-6765-3469)
- Martin van der Laan (ORCID: https://orcid.org/0000-0003-0789-9912)
- Selina Wrublewsky
- Caroline Bickelmann
- Laurie-Anne de Selliers
- Johanne Audouze-Chaud
Institutions
- Centre National de la Recherche Scientifique (FR)
- Inserm (FR)
- Universidade de São Paulo (BR)
- Université de Montpellier (FR)
- Comprehensive Cancer Center Mainfranken (DE)
- Universitätsklinikum Würzburg (DE)
- Ospedale Policlinico San Martino (IT)
- Saarland University (DE)
- UCLouvain (BE)
Publication Details
- Journal
- Diabetologia
- Published
- 2025-10-24
- DOI
- https://doi.org/10.1007/s00125-025-06575-4
- Primary Topic
- Pancreatic function and diabetes
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Deutsche Forschungsgemeinschaft
- Universität des Saarlandes