Corrigendum
Open Access | 
10.1172/jci.insight.206548
Corrigendum to: Mitochondrial bioenergetics and cardiolipin remodeling abnormalities in mitochondrial trifunctional protein deficiency
Eduardo Vieira Neto, Meicheng Wang, Austin J. Szuminsky, Lethicia Ferraro, Erik Koppes, Yudong Wang, Clinton Van’t Land, Al-Walid Mohsen, Geancarlo Zanatta, Areeg H. El-Gharbawy, Tamil S. Anthonymuthu, Yulia Y. Tyurina, Vladimir A. Tyurin, Valerian Kagan, Hülya Bayır, and Jerry Vockley
Find articles by Vieira Neto, E. in: PubMed | Google Scholar
Find articles by Wang, M. in: PubMed | Google Scholar
Find articles by Szuminsky, A. in: PubMed | Google Scholar
Find articles by Ferraro, L. in: PubMed | Google Scholar
Find articles by
Koppes, E.
in:
PubMed
|
Google Scholar
|
Find articles by Wang, Y. in: PubMed | Google Scholar
Find articles by Van’t Land, C. in: PubMed | Google Scholar
Find articles by Mohsen, A. in: PubMed | Google Scholar
Find articles by Zanatta, G. in: PubMed | Google Scholar
Find articles by El-Gharbawy, A. in: PubMed | Google Scholar
Find articles by
Anthonymuthu, T.
in:
PubMed
|
Google Scholar
|
Find articles by Tyurina, Y. in: PubMed | Google Scholar
Find articles by
Tyurin, V.
in:
PubMed
|
Google Scholar
|
Find articles by
Kagan, V.
in:
PubMed
|
Google Scholar
|
Find articles by Bayır, H. in: PubMed | Google Scholar
Find articles by
Vockley, J.
in:
PubMed
|
Google Scholar
|
Published April 8, 2026 - More info
JCI Insight. 2026;11(7):e206548. https://doi.org/10.1172/jci.insight.206548.
© 2026 Neto et al. This work is licensed under the Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
Related article:
Abstract
Mitochondrial trifunctional protein (TFP) deficiency is an inherited metabolic disorder leading to a block in long-chain fatty acid β-oxidation. Mutations in HADHA and HADHB, which encode the TFP α and β subunits, respectively, usually result in combined TFP deficiency. A single common mutation, HADHA c.1528G>C (p.E510Q), leads to isolated 3-hydroxyacyl-CoA dehydrogenase deficiency. TFP also catalyzes a step in the remodeling of cardiolipin (CL), a phospholipid critical to mitochondrial membrane stability and function. We explored the effect of mutations in TFP subunits on CL and other phospholipid content and composition and the consequences of these changes on mitochondrial bioenergetics in patient-derived fibroblasts. Abnormalities in these parameters varied extensively among different fibroblasts, and some cells were able to maintain basal oxygen consumption rates similar to controls. Although CL reduction was universally identified, a simultaneous increase in monolysocardiolipins was discrepant among cells. A similar profile was seen in liver mitochondria isolates from a TFP-deficient mouse model. Response to new potential drugs targeting CL metabolism might be dependent on patient genotype.
Authors
Eduardo Vieira Neto, Meicheng Wang, Austin J. Szuminsky, Lethicia Ferraro, Erik Koppes, Yudong Wang, Clinton Van’t Land, Al-Walid Mohsen, Geancarlo Zanatta, Areeg H. El-Gharbawy, Tamil S. Anthonymuthu, Yulia Y. Tyurina, Vladimir A. Tyurin, Valerian Kagan, Hülya Bayir, Jerry Vockley
Original citation: JCI Insight. 2024;9(17):e176887. https://doi.org/10.1172/jci.insight.176887
Citation for this corrigendum: JCI Insight. 2026;11(7):e206548. https://doi.org/10.1172/jci.insight.206548
After publication, the authors became aware of errors in figure legends, figure panel callouts, statistical comparison descriptions, and figure labels. In the legend for Figure 4E, the allele-specific expression descriptions in panels E–H were incorrect. In the Results section, under the heading “Expression of variant mRNA,” the original callouts for Figures 4A, 4B, 4E, and 4F were incorrect. The legends for Figure 7 and Figure 10 incorrectly specified the control group used for statistical comparisons as FB826, a control cell line from a 40-year-old woman. Patient numbering in Supplemental Table 2 was incorrect. Finally, the HADHA and HADHB groups were incorrectly labeled in Figure 7. The authors declare that these errors do not affect the results, interpretation, or conclusions of the study. The online HTML and PDF versions of the manuscript and the Supplemental Data Values file have been updated to correct these issues.
The authors regret the errors.
