Evidence map›Paper›PMID 32045933›Full record

ArticlePediatric research2020

Increased antioxidant response in medium-chain acyl-CoA dehydrogenase deficiency: does lipoic acid have a protective role?

Zahra Nochi, Rune Isak Dupont Birkler, Paula Fernandez-Guerra, Jakob Hansen, Flemming Wibrand, Thomas Juhl Corydon, Niels Gregersen, Rikke Katrine Jentoft Olsen

Abstract read
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Article in Pediatric research, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
0.6field-weighted citation impact, top 35% 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

1 citing paper in PubMed, 8 citations in OpenAlex.

  1. 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

8 authors at 3 institutions in 1 country.

Zahra NochiResearch Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University Hospital and Faculty of Health, Aarhus University, Aarhus, Denmark. z.nochi@clin.au.dk.
Rune Isak Dupont BirklerResearch Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University Hospital and Faculty of Health, Aarhus University, Aarhus, Denmark.
Paula Fernandez-GuerraResearch Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University Hospital and Faculty of Health, Aarhus University, Aarhus, Denmark.
Jakob HansenDepartment of Forensic Medicine, Aarhus University, Aarhus, Denmark.
Flemming WibrandDepartment of Clinical Genetics, Rigshospitalet, Copenhagen, Denmark.
Thomas Juhl CorydonDepartment of Biomedicine, Aarhus University, Aarhus, Denmark.
Niels GregersenResearch Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University Hospital and Faculty of Health, Aarhus University, Aarhus, Denmark.
Rikke Katrine Jentoft OlsenResearch Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University Hospital and Faculty of Health, Aarhus University, Aarhus, Denmark.
Aarhus University Hospital · DKAarhus University · DKRigshospitalet · DK

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundMedium-chain acyl-CoA dehydrogenase (MCAD) deficiency (MCADD) is the most frequent fatty acid oxidation (FAO) defect in humans. MCAD-deficient fibroblasts are more resistant to oxidative stress-induced cell death than other FAO defects and healthy controls.

methodsHerein we investigate the antioxidant response and mitochondrial function in fibroblasts from MCAD-deficient patients (c.985 A>G/c.985 A>G) and healthy controls.

resultsMCAD-deficient fibroblasts showed increased level of mitochondrial superoxide, while lipids were less oxidatively damaged, and higher amount of manganese superoxide dismutase were detected compared to healthy controls, showing forceful antioxidant system in MCADD. We showed increased maximal respiration and reserve capacity in MCAD-deficient fibroblasts compared to controls, indicating more capacity through the tricarboxylic acid (TCA) cycle and subsequently respiratory chain. This led us to study the pyruvate dehydrogenase complex (PDC), the key enzyme in the glycolysis releasing acetyl-CoA to the TCA cycle. MCAD-deficient fibroblasts displayed not only significantly increased PDC but also increased lipoylated PDC protein levels compared to healthy controls.

conclusionsBased on these findings, we raise the interesting hypothesis that increased PDC-bound lipoic acid, synthesized from accumulated octanoic acid in MCADD, may affect the cellular antioxidant pool in MCADD.

Indexed as

Acyl-CoA DehydrogenaseAntioxidantsCaprylatesCarnitineCell DeathFibroblastsGenotypeGlycolysisHumansLipid Metabolism, Inborn ErrorsLipid PeroxidationMitochondriaOxidative StressPhenotypeSuperoxidesThioctic AcidacylcarnitineAcyl-CoA DehydrogenaseAntioxidantsCaprylatesCarnitineoctanoic acidSuperoxidesThioctic Acid

Identifiers

PMID32045933
OpenAlexW3006191706

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

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.