Cited by 53Enzyme Structure and FunctionMitochondrial Function and PathologyBiochemical Acid Research Studies

Structural basis for malfunction in complex II

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Iverson, Tina M., Maklashina, Elena, Cecchini, Gary·2012·Journal of Biological Chemistry

Abstract

Complex II couples oxidoreduction of succinate and fumarate at one active site with that of quinol/quinone at a second distinct active site over 40 Å away. This process links the Krebs cycle to oxidative phosphorylation and ATP synthesis. The pathogenic mutation or inhibition of human complex II or its assembly factors is often associated with neurodegeneration or tumor formation in tissues derived from the neural crest. This brief overview of complex II correlates the clinical presentations of a large number of symptom-associated alterations in human complex II activity and assembly with the biochemical manifestations of similar alterations in the complex II homologs from Escherichia coli. These analyses provide clues to the molecular basis for diseases associated with aberrant complex II function.

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Crossref · 10.1074/jbc.r112.408419

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Authors
Iverson, T. M., Maklashina, E., & Cecchini, G.
Affiliations
  • Vanderbilt University Medical Center (US)
  • San Francisco VA Medical Center (US)
Corresponding author
  • Iverson, Tina M.
  • Cecchini, Gary
Venue
Journal of Biological Chemistry
Publisher
Elsevier BV
Published
2012-10
Volume
287
Issue
42
Pages
35430-35438
ISSN
0021-9258 (ISSN-L)
OpenAlex
W2029913956
Cited by
53
References
83
Citations by year
2026: 2 · 2025: 4 · 2024: 4 · 2023: 3 · 2022: 4 · 2021: 3 · 2020: 4 · 2019: 7 · 2018: 3 · 2017: 1 · 2016: 1 · 2015: 4 · 2014: 4 · 2013: 7 · 2012: 1
FWCI
0.21
Type
journal article
Language
en
Open access
Locations
  • Journal of Biological Chemistry (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • U.S. Department of Veterans Affairs (I01 BX001077)
  • National Institute of General Medical Sciences (GM79419, GM61606, R01GM061606, R01GM079419)
Topics
  • Physical Sciences › Materials Science › Materials Chemistry › Enzyme Structure and Function
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Biochemical Acid Research Studies
Keywords
complex II, mitochondrial electron transport chain, pathogenic variants, neurodegeneration, neural crest tumors, oxidative phosphorylation, TCA cycle
MeSH terms
Adenosine Triphosphate, Animals, Citric Acid Cycle, Escherichia coli, Humans, Phosphorylation, Structure-Activity Relationship, Escherichia coli Proteins, Electron Transport Complex II

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