Cited by 181Metabolism and Genetic DisordersMitochondrial Function and PathologyGlycogen Storage Diseases and Myoclonus

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Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death

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Ibdah, Jamal A., Paul, Hyacinth, Zhao, Yiwen, Binford, Scott, Salleng, Ken, Cline, Mark, Matern, Dietrich, Bennett, Michael J., Rinaldo, Piero, Strauss, Arnold W.·2001·Journal of Clinical Investigation

DOI 10.1172/jci12590PMC PMC209324

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Abstract

Mitochondrial trifunctional protein (MTP) is a hetero-octamer of four alpha and four beta subunits that catalyzes the final three steps of mitochondrial long chain fatty acid beta-oxidation. Human MTP deficiency causes Reye-like syndrome, cardiomyopathy, or sudden unexpected death. We used gene targeting to generate an MTP alpha subunit null allele and to produce mice that lack MTP alpha and beta subunits. The Mtpa(-/-) fetuses accumulate long chain fatty acid metabolites and have low birth weight compared with the Mtpa(+/-) and Mtpa(+/+) littermates. Mtpa(-/-) mice suffer neonatal hypoglycemia and sudden death 6-36 hours after birth. Analysis of the histopathological changes in the Mtpa(-/-) pups revealed rapid development of hepatic steatosis after birth and, later, significant necrosis and acute degeneration of the cardiac and diaphragmatic myocytes. This mouse model documents that intact mitochondrial long chain fatty acid oxidation is essential for fetal development and for survival after birth. Deficiency of MTP causes fetal growth retardation, neonatal hypoglycemia, and sudden death.

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Authors: Ibdah, J. A., Paul, H., Zhao, Y., Binford, S., Salleng, K., Cline, M., Matern, D., Bennett, M. J., Rinaldo, P., & Strauss, A. W.
Affiliations: Wake Forest University Health Sciences (US)
Wake Forest University (US)
Mayo Clinic (US)
The University of Texas Southwestern Medical Center (US)
Vanderbilt University (US)
Corresponding author: Ibdah, Jamal A.
ORCID: Ibdah, Jamal A.
Zhao, Yiwen
Matern, Dietrich
Bennett, Michael J.
Strauss, Arnold W.
Venue: Journal of Clinical Investigation
Publisher: American Society for Clinical Investigation
Published: 2001-06-01
Volume: 107
Issue: 11
Pages: 1403-1409
ISSN: 0021-9738 (ISSN-L)
DOI: 10.1172/jci12590
PMC: PMC209324
OpenAlex: W2144783460
Cited by: 181
References: 27
Citations by year: 2026: 2 · 2025: 4 · 2024: 7 · 2023: 4 · 2022: 5 · 2021: 5 · 2020: 11 · 2019: 7 · 2018: 5 · 2017: 10 · 2016: 9 · 2015: 12 · 2014: 5 · 2013: 9 · 2012: 6
FWCI: 4.46
Type: journal article
Language: en
Open access: bronze
doi.org
Locations: Journal of Clinical Investigation (publishedVersion)
Europe PMC (PubMed Central) (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Funders: National Institutes of Health (AM 20407, 5k08dk002574-04)
National Institute of Diabetes and Digestive and Kidney Diseases (DK-02574)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Glycogen Storage Diseases and Myoclonus
Keywords: mitochondrial trifunctional protein, long-chain fatty acid beta-oxidation, neonatal hypoglycemia, sudden unexpected death, hepatic steatosis, myocardial necrosis, fetal growth restriction, Reye-like syndrome, mitochondrial fatty acid oxidation
MeSH terms: Animals, Animals, Newborn, Blood Chemical Analysis, Crosses, Genetic, Death, Sudden, Diaphragm, Disease Models, Animal, Female, Embryonic and Fetal Development, Fetal Growth Retardation, Humans, Hypoglycemia, Liver, Male, Multienzyme Complexes, Myocardium, Immunoblotting, Mice, Knockout, Gene Targeting, Mice, Mitochondrial Trifunctional Protein

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Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death

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