Evidence map›Paper›PMID 34923139›Full record

ArticleThe international journal of biochemistry & cell biology2022

The dynamin-related protein 1 is decreased and the mitochondrial network is altered in Friedreich's ataxia cardiomyopathy.

Bojjibabu Chidipi, Mariana Burgos Angulo, Syed Islamuddin Shah, Michelle Rieser, Ganim Ullah, Thomas V McDonald, Sami F Noujaim

Open access · greenAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in The international journal of biochemistry & cell biology, 2022. 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
0.3field-weighted citation impact, top 43% of its field
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, 5 citations in OpenAlex.

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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

7 authors at 1 institution in 1 country.

Bojjibabu ChidipiDepartment of Molecular Pharmacology and Physiology, Morsani College of Medicine, University of South Florida, Tampa, FL, USA. Electronic address: chidipib@usf.edu.
Mariana Burgos AnguloDepartment of Molecular Pharmacology and Physiology, Morsani College of Medicine, University of South Florida, Tampa, FL, USA; Department of Medicine (Cardiology), Morsani College of Medicine, University of South Florida, Tampa, FL, USA.
Syed Islamuddin ShahDepartment of Physics, University of South Florida, Tampa, FL, USA.
Michelle RieserDepartment of Molecular Pharmacology and Physiology, Morsani College of Medicine, University of South Florida, Tampa, FL, USA.
Ganim UllahDepartment of Physics, University of South Florida, Tampa, FL, USA.
Thomas V McDonaldDepartment of Molecular Pharmacology and Physiology, Morsani College of Medicine, University of South Florida, Tampa, FL, USA; Department of Medicine (Cardiology), Morsani College of Medicine, University of South Florida, Tampa, FL, USA.
Sami F NoujaimDepartment of Molecular Pharmacology and Physiology, Morsani College of Medicine, University of South Florida, Tampa, FL, USA.
University of South Florida · US

Funding

A multi-scale data-driven model of the Abeta pore function and Ca2+ toxicity in Alzheimer's diseaseR01AG053988 · NIA · UNIVERSITY OF SOUTH FLORIDA · PI DEMURO, ANGELO, ULLAH, GHANIM · 2016 to 2020
$2.1M
Molecular Mechanisms for Atrial Fibrillation in AgingR01HL129136 · NHLBI · UNIVERSITY OF SOUTH FLORIDA · PI NOUJAIM, SAMI FOUAD · 2015 to 2019
$2.0M
NHLBI NIH HHS R01 HL129136NIA NIH HHS R01 AG053988
6 · The paper itself

Abstract

Friedreich ataxia is an autosomal recessive congenital neurodegenerative disease caused by a deficiency in the frataxin protein and is often diagnosed in young adulthood. An expansion of guanine-adenine-adenine repeats in the first intron of the FXN gene leads to decreased frataxin expression. Frataxin plays an essential role in mitochondrial metabolism. Most Friedreich ataxia patients are diagnosed with left ventricular hypertrophic cardiomyopathy, and 60% of patients die with hypertrophic cardiomyopathy. However, the mitochondrial anatomy in Friedreich ataxia hypertrophic cardiomyopathy is still poorly understood. We investigated mitochondrial fission, fusion, and function using biochemical, microscopy, and computational stochastic analysis in human induced pluripotent stem cell derived cardiomyocytes from a patient with Friedreich ataxia hypertrophic cardiomyopathy and a healthy individual. We found a significantly higher mitochondrial footprint, decreased mitochondrial fission protein dynamin-related protein, and mitochondrial fission rate over fusion with more giant mitochondrial clusters in human induced pluripotent stem cell derived cardiomyocytes from a patient with Friedreich ataxia hypertrophic cardiomyopathy, compared to an unaffected individual. We also found significantly depolarized mitochondrial membrane potential and higher reactive oxygen species levels in Friedreich ataxia human induced pluripotent stem cell cardiomyocytes. Our results show that frataxin's depletion may dampen the mitochondrial fission machinery by reducing dynamin-related protein1. The loss of mitochondrial fission might lead to elevated reactive oxygen species and depolarized mitochondrial membrane potential, which may cause oxidative damage in Friedreich ataxia hypertrophic cardiomyopathy. Further investigations are needed to identify the mechanism of downregulating dynamin-related protein1 due to the frataxin deficiency in Friedreich ataxia hypertrophic cardiomyopathy.

Indexed as

AdolescentCardiomyopathy, HypertrophicChildDynaminsFemaleFriedreich AtaxiaHumansMaleMitochondriaNeurodegenerative DiseasesDNM1L protein, humanDynaminsDRP1Friedreich ataxiaHypertrophic cardiomyopathyMitochondrial fusion and fission

Identifiers

PMID34923139
PMCPMC8820922
OpenAlexW4200399115

What OpenQuestion holds

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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 OpenQuestion graph.