ArticlePloS one2012
Compromised mitochondrial fatty acid synthesis in transgenic mice results in defective protein lipoylation and energy disequilibrium.
Article in PloS one, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
What it found
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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.
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Who cites it
35 citing papers in PubMed, 49 citations in OpenAlex.
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- Abcb5-deficient mice show a subtle, pleiotropic phenotype indicating a role for this transporter in intermediary metabolism.iScience · 2025Article
- A mouse model of MEPAN demonstrates a role for mitochondrial fatty acid synthesis in iron-sulfur cluster and supercomplex formation.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Mitochondrial fatty acid synthesis is an emergent central regulator of mammalian oxidative metabolism.Cell metabolism · 2024Review
- Autosomal recessive Leber hereditary optic neuropathy, a new neuro-ophthalmo-genetic paradigm.Brain : a journal of neurology · 2023Article
- Recessive pathogenic variants ineLife · 2023Article
- Measurement of lipogenic flux by deuterium resolved mass spectrometry.Nature communications · 2021Article
- Article
- The Role of Mitochondria in Optic Atrophy With Autosomal Inheritance.Frontiers in neuroscience · 2021Review
- Role of Metabolism in Bone Development and Homeostasis.International journal of molecular sciences · 2020Review
- Mitochondrial fatty acid synthesis coordinates oxidative metabolism in mammalian mitochondria.eLife · 2020Article
- Progress in the Enzymology of the Mitochondrial Diseases of Lipoic Acid Requiring Enzymes.Frontiers in genetics · 2020Review
- Role of the malonyl-CoA synthetase ACSF3 in mitochondrial metabolism.Advances in biological regulation · 2019Review
- Impaired Mitochondrial Fatty Acid Synthesis Leads to Neurodegeneration in Mice.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2018Article
- Protein moonlighting elucidates the essential human pathway catalyzing lipoic acid assembly on its cognate enzymes.Proceedings of the National Academy of Sciences of the United States of America · 2018Article
- Lipoic acid metabolism and mitochondrial redox regulation.The Journal of biological chemistry · 2018Review
- Identifying off-target effects of etomoxir reveals that carnitine palmitoyltransferase I is essential for cancer cell proliferation independent of β-oxidation.PLoS biology · 2018Article
- Protein lipoylation: an evolutionarily conserved metabolic regulator of health and disease.Current opinion in chemical biology · 2018Review
- Regulation of malonyl-CoA-acyl carrier protein transacylase network in umbilical cord blood affected by intrauterine hyperglycemia.Oncotarget · 2017Article
- Role of S-Palmitoylation by ZDHHC13 in Mitochondrial function and Metabolism in Liver.Scientific reports · 2017Article
Corrections and comments
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Authors and funding
28 authors at 5 institutions in 1 country.
Funding
Abstract
A mouse model with compromised mitochondrial fatty acid synthesis has been engineered in order to assess the role of this pathway in mitochondrial function and overall health. Reduction in the expression of mitochondrial malonyl CoA-acyl carrier protein transacylase, a key enzyme in the pathway encoded by the nuclear Mcat gene, was achieved to varying extents in all examined tissues employing tamoxifen-inducible Cre-lox technology. Although affected mice consumed more food than control animals, they failed to gain weight, were less physically active, suffered from loss of white adipose tissue, reduced muscle strength, kyphosis, alopecia, hypothermia and shortened lifespan. The Mcat-deficient phenotype is attributed primarily to reduced synthesis, in several tissues, of the octanoyl precursors required for the posttranslational lipoylation of pyruvate and α-ketoglutarate dehydrogenase complexes, resulting in diminished capacity of the citric acid cycle and disruption of energy metabolism. The presence of an alternative lipoylation pathway that utilizes exogenous free lipoate appears restricted to liver and alone is insufficient for preservation of normal energy metabolism. Thus, de novo synthesis of precursors for the protein lipoylation pathway plays a vital role in maintenance of mitochondrial function and overall vigor.
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What OpenQuestion holds
Registered trials
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.