Evidence mapPaperPMID 40691304Full record

ArticleNature metabolism2025

LONP1 regulation of mitochondrial protein folding provides insight into beta cell failure in type 2 diabetes.

Jin Li, Yamei Deng, Marie Gasser, Jie Zhu, Emily M Walker, Vaibhav Sidarala, Emma C Reck, Dre L Hubers, Mabelle B Pasmooij, Chun-Shik Shin and 10 more

Abstract read
In one paragraph

Article in Nature metabolism, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.

0numbers the graph read from it
0cells of the map it votes in
14citing papers in PubMed
field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

14 citing papers in PubMed.

  1. Article
  2. Review
  3. Review
  4. Article
  5. Review
  6. Review
  7. Mitochondria-associated programmed cell death in pancreatic β cell of T2DM.Apoptosis : an international journal on programmed cell death · 2026
    Review
  8. Molecular mechanisms of mitochondrial AAA+ proteases.The Journal of biological chemistry · 2026
    Review
  9. Review
  10. Article
  11. Review
  12. Article
  13. Article
  14. Article
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

20 authors.

Jin LiDivision of Metabolism, Endocrinology & Diabetes and Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Yamei DengDepartment of Pathology, University of Michigan, Ann Arbor, MI, USA.ORCID http://orcid.org/0000-0002-3680-5930
Marie GasserCR-CHUM, University of Montreal, Montreal, Quebec, Canada.
Jie ZhuDivision of Metabolism, Endocrinology & Diabetes and Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Emily M WalkerDivision of Metabolism, Endocrinology & Diabetes and Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.ORCID http://orcid.org/0000-0001-8394-1567
Vaibhav SidaralaDivision of Metabolism, Endocrinology & Diabetes and Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Emma C ReckDivision of Metabolism, Endocrinology & Diabetes and Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Dre L HubersDivision of Metabolism, Endocrinology & Diabetes and Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Mabelle B PasmooijDivision of Metabolism, Endocrinology & Diabetes and Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Chun-Shik ShinDivision of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA, USA.
Khushdeep BandeshThe Jackson Laboratory for Genomic Medicine, Farmington, CT, USA.ORCID http://orcid.org/0000-0002-5057-7313
Eftyhmios MotakisThe Jackson Laboratory for Genomic Medicine, Farmington, CT, USA.
Siddhi NargundThe Jackson Laboratory for Genomic Medicine, Farmington, CT, USA.
Romy KursaweThe Jackson Laboratory for Genomic Medicine, Farmington, CT, USA.
Venkatesha BasrurDepartment of Pathology, University of Michigan, Ann Arbor, MI, USA.
Alexey I NesvizhskiiDepartment of Pathology, University of Michigan, Ann Arbor, MI, USA.ORCID http://orcid.org/0000-0002-2806-7819
Michael L StitzelThe Jackson Laboratory for Genomic Medicine, Farmington, CT, USA.ORCID http://orcid.org/0000-0001-5630-559X
David C ChanDivision of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA, USA.ORCID http://orcid.org/0000-0002-0191-2154
Guy A RutterCR-CHUM, University of Montreal, Montreal, Quebec, Canada.
Scott A SoleimanpourDivision of Metabolism, Endocrinology & Diabetes and Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. ssol@med.umich.edu.ORCID http://orcid.org/0000-0001-6777-4498

Funding

Regional Pilot And Feasibility Study Grants ProgramP30DK020572 · NIDDK · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI Scott Soleimanpour · 2013 to 2026
$24.3M
Integrated Islet Distribution Program (U24) - 2021U24DK098085 · NIDDK · BECKMAN RESEARCH INSTITUTE/CITY OF HOPE · PI Carmella Evans-Molina, Joyce Carol Niland · 2021 to 2026
$17.8M
Homeostatic Mechanisms Regulating Mitochondrial Health EQUIPMENT SUPPLEMENTR35GM127147 · NIGMS · CALIFORNIA INSTITUTE OF TECHNOLOGY · PI David C Chan · 2018 to 2026
$6.0M
COMPUTATIONAL TOOLS FOR MASS SPECTROMETRY-BASED INTERACTOME DATAR01GM094231 · NIGMS · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI Alexey I Nesvizhskii · 2010 to 2026
$5.4M
Genetic programming of human islet metabolic and endoplasmic reticulum (ER) stress responses in diabetesR01DK118011 · NIDDK · JACKSON LABORATORY · PI Michael Lee Stitzel · 2021 to 2026
$4.9M
Type 2 diabetes risk variant effects on mitochondrial (patho)physiologyR01DK136671 · NIDDK · JACKSON LABORATORY · PI Scott Soleimanpour, Michael Lee Stitzel · 2023 to 2026
$3.0M
Functional interaction of transcriptional regulators in endocrine lineage specificationR01DK135032 · NIDDK · UNIVERSITY OF PENNSYLVANIA · PI Maureen A Gannon, DORIS A STOFFERS · 2023 to 2026
$2.8M
A Stress-Induced Vicious Cycle In The Development of T1DU01DK127747 · NIDDK · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI ARVAN, PETER, SATIN, LESLIE S. · 2020 to 2023
$2.8M
Control of insulin secretion by mitochondrial fusionR01DK135268 · NIDDK · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI Brett A Kaufman, Scott Soleimanpour · 2023 to 2026
$2.4M
Structure dictates dynamic topology and function of pancreatic transcriptional regulatorsR01DK127270 · NIDDK · UNIVERSITY OF PENNSYLVANIA · PI Maureen A Gannon, DORIS A STOFFERS · 2024 to 2026
$2.0M
Mediators of mitophagy in the regulation of beta cell functionR01DK108921 · NIDDK · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI SOLEIMANPOUR, SCOTT · 2016 to 2020
$1.9M
Promoting metabolic maturity of islet organoids pre- and post-transplantationR01DK142799 · NIDDK · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI Sriram Chandrasekaran, Lonnie D Shea · 2025 to 2026
$1.4M
American Diabetes Association (ADA) 1-25-PDF-126American Diabetes Association (ADA) 1-81-ACE-015BLRD VA I01 BX004444Diabetes UK BDA 16/0005485Gouvernement du Canada | Instituts de Recherche en Santé du Canada | CIHR Skin Research Training Centre (Skin Research Training Centre) CIHR-IRSC TDP-186358Innovation, Science and Economic Development Canada CFI 42649JDRF SRA-2023-1392, SRA-2024-1586NIDDK NIH HHS K01 DK133533NIDDK NIH HHS P30 DK020572NIDDK NIH HHS R01 DK108921NIDDK NIH HHS R01 DK118011NIDDK NIH HHS R01 DK127270NIDDK NIH HHS R01 DK135032NIDDK NIH HHS R01 DK135268NIDDK NIH HHS R01 DK136671NIDDK NIH HHS R01 DK142799NIDDK NIH HHS U01 DK127747NIDDK NIH HHS U24 DK098085NIGMS NIH HHS R01 GM094231NIGMS NIH HHS R35 GM127147RCUK | Medical Research Council (MRC) MR/R022259/1Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung (Swiss National Science Foundation) P500PM_225305 / 1U.S. Department of Health & Human Services | NIH | National Institute of Diabetes and Digestive and Kidney Diseases (National Institute of Diabetes & Digestive & Kidney Diseases) R01 DK108921, R01 DK135032, R01 DK135268, R01 DK136671, R01 DK127270, U01 DK127747, P30 DK020572U.S. Department of Health & Human Services | NIH | National Institute of Diabetes and Digestive and Kidney Diseases (National Institute of Diabetes & Digestive & Kidney Diseases) R01DK135268U.S. Department of Health & Human Services | NIH | National Institute of Diabetes and Digestive and Kidney Diseases (National Institute of Diabetes & Digestive & Kidney Diseases) R01 DK136671, R01 DK118011Wellcome TrustWellcome Trust (Wellcome) WT212625/Z/18/Z
6 · The paper itself

Abstract

Protein misfolding is a contributor to the development of type 2 diabetes (T2D), but the specific role of impaired proteostasis is unclear. Here we show a robust accumulation of misfolded proteins in the mitochondria of human pancreatic islets from patients with T2D and elucidate its impact on β cell viability through the mitochondrial matrix protease LONP1. Quantitative proteomics studies of protein aggregates reveal that islets from donors with T2D have a signature resembling mitochondrial rather than endoplasmic reticulum protein misfolding. Loss of LONP1, a vital component of the mitochondrial proteostatic machinery, with reduced expression in the β cells of donors with T2D, yields mitochondrial protein misfolding and reduced respiratory function, leading to β cell apoptosis and hyperglycaemia. LONP1 gain of function ameliorates mitochondrial protein misfolding and restores human β cell survival after glucolipotoxicity via a protease-independent effect requiring LONP1-mitochondrial HSP70 chaperone activity. Thus, LONP1 promotes β cell survival and prevents hyperglycaemia by facilitating mitochondrial protein folding. These observations provide insights into the nature of proteotoxicity that promotes β cell loss during the pathogenesis of T2D, which could be considered as future therapeutic targets.

Indexed as

ATP-Dependent ProteasesDiabetes Mellitus, Type 2Insulin-Secreting CellsMitochondriaMitochondrial ProteinsProtein FoldingApoptosisCell SurvivalHumansATP-Dependent ProteasesLONP1 protein, humanMitochondrial Proteins

Identifiers

PMID40691304
PMCPMC12373512

What Socratic holds

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LicenceCC BY-NC-ND
Read underepoch 390

Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.