The DOI matches its Crossref record; 32 of the 32 references it cites were found in a registry or an index.
Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase
Abstract
The Sod2 gene for Mn-superoxide dismutase (MnSOD), an intramitochondrial free radical scavenging enzyme that is the first line of defense against superoxide produced as a byproduct of oxidative phosphorylation, was inactivated by homologous recombination. Homozygous mutant mice die within the first 10 days of life with a dilated cardiomyopathy, accumulation of lipid in liver and skeletal muscle, and metabolic acidosis. Cytochemical analysis revealed a severe reduction in succinate dehydrogenase (complex II) and aconitase (a TCA cycle enzyme) activities in the heart and, to a lesser extent, in other organs. These findings indicate that MnSOD is required for normal biological function of tissues by maintaining the integrity of mitochondrial enzymes susceptible to direct inactivation by superoxide.
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In the citing paper
“Primary mechanisms of nitrated fatty acid signaling include activation of PPARγ (55, 583) and Michael addition to thiol-containing proteins in a number of signaling pathways (53, 955).”
From Sources of Vascular Nitric Oxide and Reactive Oxygen Species and Their Regulation (2019) · cites this paper as [583]
In the citing paper
“Its deficiency causes early neonatal death in knockout mice, which suffer from dilated cardiomyopathy and neurodegeneration (236, 252).”
From Redox regulation of mitochondrial function (2012) · cites this paper as [252]
In the citing paper
“Sod2−/− mice die before birth”
From Mitochondrial reactive oxygen species regulate cellular signaling and dictate biological outcomes (2010) · cites this paper as [88]
In the citing paper
“their heterozygous littermates exhibit mitochondrial damage and oxidative modifications to both nuclear and mitochondrial DNA”
From Mitochondrial reactive oxygen species regulate cellular signaling and dictate biological outcomes (2010) · cites this paper as [88]
In the citing paper
“However, these heterozygous mice have a normal lifespan 88, 89.”
From Mitochondrial reactive oxygen species regulate cellular signaling and dictate biological outcomes (2010) · cites this paper as [88]
Citations
Cites
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Self-citationKu, H., Brunk, U. T., & Sohal, R. S. (1993) · Free Radical Biology and Medicine · doi:10.1016/0891-5849(93)90165-q · Cited by 488
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Superoxide Radical and Iron Modulate Aconitase Activity in Mammalian Cells
Gardner, P. R., Raineri, I., Epstein, L. B., & White, C. W. (1995) · Journal of Biological Chemistry · doi:10.1074/jbc.270.22.13399 · Cited by 469
Flagged first, then by citations
Research works printed but not linked: 1
- Fridovich, I. Measuring the activity of superoxide dismutase: an embarrassment of riches. In Superoxide Dismutase 1 (ed. Oberley, C.W.) 69–77 (CRC Press, Boca Raton 1982).
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Items 1,001–1,002 of 1,002
Long chain fatty acyl-CoA modulation of H2O2 release at mitochondrial complex I
Bortolami, S., Comelato, E., Zoccarato, F., Alexandre, A., & Cavallini, L. (2008) · Journal of Bioenergetics and Biomembranes · doi:10.1007/s10863-008-9126-1 · Cited by 13
The control of mitochondrial succinate-dependent H2O2 production
Zoccarato, F., Miotto, C., Cavallini, L., & Alexandre, A. (2011) · Journal of Bioenergetics and Biomembranes · doi:10.1007/s10863-011-9363-6 · Cited by 8
Metadata
- Authors
- Li, Y., Huang, T.-T., Carlson, E. J., Melov, S., Ursell, P. C., Olson, J. L., Noble, L. J., Yoshimura, M. P., Berger, C., Chan, P. H., Wallace, D. C., & Epstein, C. J.
- Affiliations
- University of California, San Francisco (US)
- Emory University (US)
- Children's Medical Research Institute (AU)
- Venue
- Nature Genetics
- Published
- 1995-12
- Volume
- 11
- Issue
- 4
- Pages
- 376-381
- ISSN
- 1061-4036 (ISSN-L)
- 1546-1718
- OpenAlex
- W2094655287
- Cited by
- 1,789
- References
- 32
- Citations by year
- 2026: 20 · 2025: 35 · 2024: 23 · 2023: 31 · 2022: 32 · 2021: 48 · 2020: 39 · 2019: 36 · 2018: 42 · 2017: 52 · 2016: 58 · 2015: 62 · 2014: 102 · 2013: 87 · 2012: 101
- FWCI
- 9.79
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Genetics (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute on Aging (P30AG010130, AG-10130, AG-13154, AG-08938, P01AG008938, R01AG013154)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Redox biology and oxidative stress
- Keywords
- manganese superoxide dismutase, dilated cardiomyopathy, neonatal lethality, mitochondrial superoxide, oxidative phosphorylation, succinate dehydrogenase, complex II, aconitase, TCA cycle, lipid accumulation, metabolic acidosis, Sod2 knockout mice
- MeSH terms
- Acidosis, Animals, Animals, Newborn, Base Sequence, Cardiomyopathy, Dilated, Electron Transport Complex IV, Genes, Lethal, Homozygote, Lipids, Liver, Mice, Mutant Strains, Mitochondria, Heart, Mitochondria, Muscle, Molecular Sequence Data, Succinate Dehydrogenase, Superoxide Dismutase, Lipid Peroxidation, Sequence Deletion, Gene Targeting, Muscle, Skeletal, Mice
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