Evidence map›Paper›PMID 39509858›Full record

ArticleBiomaterials2025

Rescue of mitochondrial dysfunction through alteration of extracellular matrix composition in barth syndrome cardiac fibroblasts.

Janny Piñeiro-Llanes, Silveli Suzuki-Hatano, Ananya Jain, Sree Venigalla, Manasi Kamat, Kari B Basso, William T Cade, Chelsey S Simmons, Christina A Pacak

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Biomaterials, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing 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

4 citing papers in PubMed.

  1. Article
  2. Biomimetic materials for medical applications.Chinese medical journal · 2026
    Review
  3. Article
  4. Barth Syndrome:Genes · 2025
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors.

Janny Piñeiro-LlanesJ. Crayton Pruitt Family Department of Biomedical Engineering, Herbert Wertheim College of Engineering, University of Florida, Gainesville, FL, 32611, USA. Electronic address: jpineiro@ufl.edu.
Silveli Suzuki-HatanoDepartment of Pediatrics, University of Florida College of Medicine, Gainesville, FL, 32610, USA. Electronic address: silvelisuzuki@gmail.com.
Ananya JainJ. Crayton Pruitt Family Department of Biomedical Engineering, Herbert Wertheim College of Engineering, University of Florida, Gainesville, FL, 32611, USA. Electronic address: ananyajain@ufl.edu.
Sree VenigallaDepartment of Neurology and Greg Marzolf Jr. Muscular Dystrophy Center, University of Minnesota Medical School, Minneapolis, 55455, USA. Electronic address: venig004@umn.edu.
Manasi KamatDepartment of Chemistry, University of Florida, Gainesville, FL 32611, USA. Electronic address: manasi@ufl.edu.
Kari B BassoDepartment of Chemistry, University of Florida, Gainesville, FL 32611, USA. Electronic address: kari.basso@chem.ufl.edu.
William T CadeDoctor of Physical Therapy Division, Duke University, Durham, NC, 27710, USA. Electronic address: todd.cade@duke.edu.
Chelsey S SimmonsJ. Crayton Pruitt Family Department of Biomedical Engineering, Herbert Wertheim College of Engineering, University of Florida, Gainesville, FL, 32611, USA; Department of Mechanical and Aerospace Engineering, Herbert Wertheim College of Engineering, University of Florida, Gainesville, FL, 32611, USA. Electronic address: css@ufl.edu.
Christina A PacakDepartment of Pediatrics, University of Florida College of Medicine, Gainesville, FL, 32610, USA; Department of Neurology and Greg Marzolf Jr. Muscular Dystrophy Center, University of Minnesota Medical School, Minneapolis, 55455, USA. Electronic address: cpacak@umn.edu.

Funding

Mechanisms and Treatment of Cardiac and Skeletal Muscular Dysfunction in Barth SyndromeR01HL136759 · NHLBI · UNIVERSITY OF MINNESOTA · PI PACAK, CHRISTINA A. · 2018 to 2022
$2.2M
Acquisition of a Q Exactive HF Hybrid Quadrupole-OrbitrapS10OD021758 · OD · UNIVERSITY OF FLORIDA · PI BASSO, KARI B · 2017 to 2017
$600k
NHLBI NIH HHS R01 HL136759NIH HHS S10 OD021758
6 · The paper itself

Abstract

Fibroblast-ECM (dys)regulation is associated with a plethora of diseases. The ECM acts as a reservoir of inflammatory factors and cytokines that mediate molecular mechanisms within cardiac cell populations. The role of ECM-mitochondria crosstalk in the development and progression of cardiac disorders remains uncertain. We evaluated the influence of ECM produced by stromal cells from patients with the mitochondrial cardiomyopathy (Barth syndrome, BTHS) and unaffected healthy controls on cardiac fibroblast (CF) metabolic function. To do this, cell-derived matrices CDMs were generated from BTHS and healthy human pluripotent stem cell-derived CFs (hPSC-CF) and used as cell culture substrates. BTHS CDMs negatively impacted the mitochondrial function of healthy hPSC-CFs while healthy CDMs improved mitochondrial function in BTHS hPSC-CFs. Mass spectrometry comparisons identified 5 matrisome proteins differentially expressed in BTHS compared to healthy CDM. Our results highlight a key role for the ECM in disease through its impact on mitochondrial function.

Indexed as

Barth SyndromeExtracellular MatrixFibroblastsCells, CulturedFemaleHumansMaleMitochondriaMyocardiumBarth syndromeCardiac fibroblastsCell derived matricesExtracellular matrixMitochondria

Identifiers

PMID39509858
PMCPMC11625619

What OpenQuestion holds

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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 OpenQuestion graph.