Human COQ10A and COQ10B are distinct lipid-binding START domain proteins required for coenzyme Q function
Abstract
We report that coenzyme Q (ubiquinone or CoQ) serves as an essential redox-active lipid in respiratory electron and proton transport during cellular energy metabolism. CoQ also functions as a membrane-localized antioxidant protecting cells against lipid peroxidation. CoQ deficiency is associated with multiple human diseases; CoQ10 supplementation in particular has noted cardioprotective benefits. In Saccharomyces cerevisiae, Coq10, a putative START domain protein, is believed to chaperone CoQ to sites where it functions. Yeast coq10 deletion mutants (coq10Δ) synthesize CoQ inefficiently during log phase growth, are respiratory defective and sensitive to oxidative stress. Humans have two orthologs of yeast COQ10, COQ10A and COQ10B. Here, we tested the human co-orthologs for their ability to rescue the yeast mutant. We showed that expression of either human ortholog, COQ10A or COQ10B, rescues yeast coq10Δ mutant phenotypes, restoring the function of respiratory-dependent growth on a non-fermentable carbon source and sensitivity to oxidative stress induced by treatment with polyunsaturated fatty acids. These effects indicate a strong functional conservation of Coq10 across different organisms. However, neither COQ10A nor COQ10B restored CoQ biosynthesis when expressed in the yeast coq10Δ mutant. The involvement of yeast Coq10 in CoQ biosynthesis may rely on its interactions with another protein, possibly Coq11, whichmore »
- Authors:
-
[2];
- Univ. of California, Los Angeles, CA (United States)
- Brookhaven National Lab. (BNL), Upton, NY (United States)
- Publication Date:
- Research Org.:
- Brookhaven National Lab. (BNL), Upton, NY (United States)
- Sponsoring Org.:
- USDOE Office of Science (SC), Biological and Environmental Research (BER)
- OSTI Identifier:
- 1556881
- Report Number(s):
- BNL-211981-2019-JAAM
Journal ID: ISSN 0022-2275
- Grant/Contract Number:
- SC0012704
- Resource Type:
- Journal Article: Accepted Manuscript
- Journal Name:
- Journal of Lipid Research
- Additional Journal Information:
- Journal Volume: 60; Journal Issue: 7; Journal ID: ISSN 0022-2275
- Publisher:
- American Society for Biochemistry and Molecular Biology
- Country of Publication:
- United States
- Language:
- English
- Subject:
- 59 BASIC BIOLOGICAL SCIENCES; 37 INORGANIC, ORGANIC, PHYSICAL, AND ANALYTICAL CHEMISTRY; Antioxidants; Lipids; Chemistry; Peroxidation; Mass spectrometry; Mitochondria
Citation Formats
Tsui, Hui S., Pham, Nguyen V. B., Amer, Brendan R., Bradley, Michelle C., Gosschalk, Jason E., Gallagher-Jones, Marcus, Ibarra, Hope, Clubb, Robert T., Blaby-Haas, Crysten E., and Clarke, Catherine F. Human COQ10A and COQ10B are distinct lipid-binding START domain proteins required for coenzyme Q function. United States: N. p., 2019.
Web. doi:10.1194/jlr.M093534.
Tsui, Hui S., Pham, Nguyen V. B., Amer, Brendan R., Bradley, Michelle C., Gosschalk, Jason E., Gallagher-Jones, Marcus, Ibarra, Hope, Clubb, Robert T., Blaby-Haas, Crysten E., & Clarke, Catherine F. Human COQ10A and COQ10B are distinct lipid-binding START domain proteins required for coenzyme Q function. United States. https://doi.org/10.1194/jlr.M093534
Tsui, Hui S., Pham, Nguyen V. B., Amer, Brendan R., Bradley, Michelle C., Gosschalk, Jason E., Gallagher-Jones, Marcus, Ibarra, Hope, Clubb, Robert T., Blaby-Haas, Crysten E., and Clarke, Catherine F. Thu .
"Human COQ10A and COQ10B are distinct lipid-binding START domain proteins required for coenzyme Q function". United States. https://doi.org/10.1194/jlr.M093534. https://www.osti.gov/servlets/purl/1556881.
@article{osti_1556881,
title = {Human COQ10A and COQ10B are distinct lipid-binding START domain proteins required for coenzyme Q function},
author = {Tsui, Hui S. and Pham, Nguyen V. B. and Amer, Brendan R. and Bradley, Michelle C. and Gosschalk, Jason E. and Gallagher-Jones, Marcus and Ibarra, Hope and Clubb, Robert T. and Blaby-Haas, Crysten E. and Clarke, Catherine F.},
abstractNote = {We report that coenzyme Q (ubiquinone or CoQ) serves as an essential redox-active lipid in respiratory electron and proton transport during cellular energy metabolism. CoQ also functions as a membrane-localized antioxidant protecting cells against lipid peroxidation. CoQ deficiency is associated with multiple human diseases; CoQ10 supplementation in particular has noted cardioprotective benefits. In Saccharomyces cerevisiae, Coq10, a putative START domain protein, is believed to chaperone CoQ to sites where it functions. Yeast coq10 deletion mutants (coq10Δ) synthesize CoQ inefficiently during log phase growth, are respiratory defective and sensitive to oxidative stress. Humans have two orthologs of yeast COQ10, COQ10A and COQ10B. Here, we tested the human co-orthologs for their ability to rescue the yeast mutant. We showed that expression of either human ortholog, COQ10A or COQ10B, rescues yeast coq10Δ mutant phenotypes, restoring the function of respiratory-dependent growth on a non-fermentable carbon source and sensitivity to oxidative stress induced by treatment with polyunsaturated fatty acids. These effects indicate a strong functional conservation of Coq10 across different organisms. However, neither COQ10A nor COQ10B restored CoQ biosynthesis when expressed in the yeast coq10Δ mutant. The involvement of yeast Coq10 in CoQ biosynthesis may rely on its interactions with another protein, possibly Coq11, which is not found in humans. Lastly, co-expression analyses of yeast COQ10, and human COQ10A and COQ10B provide additional insights to functions of these START domain proteins, and their potential roles in other biologic pathways.},
doi = {10.1194/jlr.M093534},
url = {https://www.osti.gov/biblio/1556881},
journal = {Journal of Lipid Research},
issn = {0022-2275},
number = 7,
volume = 60,
place = {United States},
year = {2019},
month = {5}
}
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Works referenced in this record:
Clinical syndromes associated with Coenzyme Q10 deficiency
journal, July 2018
- Alcázar-Fabra, María; Trevisson, Eva; Brea-Calvo, Gloria
- Essays in Biochemistry, Vol. 62, Issue 3
Metabolism and function of coenzyme Q
journal, January 2004
- Turunen, Mikael; Olsson, Jerker; Dallner, Gustav
- Biochimica et Biophysica Acta (BBA) - Biomembranes, Vol. 1660, Issue 1-2
Genetic bases and clinical manifestations of coenzyme Q10 (CoQ10) deficiency
journal, August 2014
- Desbats, Maria Andrea; Lunardi, Giada; Doimo, Mara
- Journal of Inherited Metabolic Disease, Vol. 38, Issue 1
Biochemistry of Mitochondrial Coenzyme Q Biosynthesis
journal, October 2017
- Stefely, Jonathan A.; Pagliarini, David J.
- Trends in Biochemical Sciences, Vol. 42, Issue 10
Coenzyme Q10 deficiencies: pathways in yeast and humans
journal, July 2018
- Awad, Agape M.; Bradley, Michelle C.; Fernández-del-Río, Lucía
- Essays in Biochemistry, Vol. 62, Issue 3
A Soluble Metabolon Synthesizes the Isoprenoid Lipid Ubiquinone
journal, April 2019
- Hajj Chehade, Mahmoud; Pelosi, Ludovic; Fyfe, Cameron David
- Cell Chemical Biology, Vol. 26, Issue 4
Polyprenyl diphosphate synthase essentially defines the length of the side chain of ubiquinone
journal, August 1996
- Okada, Kazunori; Suzuki, Kengo; Kamiya, Yasuhiro
- Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, Vol. 1302, Issue 3
Biosynthesis of coenzyme Q in eukaryotes
journal, July 2015
- Kawamukai, Makoto
- Bioscience, Biotechnology, and Biochemistry, Vol. 80, Issue 1
A conserved START domain coenzyme Q-binding polypeptide is required for efficient Q biosynthesis, respiratory electron transport, and antioxidant function in Saccharomyces cerevisiae
journal, April 2013
- Allan, Christopher M.; Hill, Shauna; Morvaridi, Susan
- Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids, Vol. 1831, Issue 4
The Saccharomyces cerevisiae COQ10 Gene Encodes a START Domain Protein Required for Function of Coenzyme Q in Respiration
journal, October 2005
- Barros, Mario H.; Johnson, Alisha; Gin, Peter
- Journal of Biological Chemistry, Vol. 280, Issue 52
NMR structure of the 18 kDa protein CC1736 from Caulobacter crescentus identifies a member of the “START” domain superfamily and suggests residues mediating substrate specificity
journal, December 2004
- Shen, Yang; Goldsmith-Fischman, Sharon; Atreya, Hanudatta S.
- Proteins: Structure, Function, and Bioinformatics, Vol. 58, Issue 3
START: a lipid-binding domain in StAR, HD-ZIP and signalling proteins
journal, April 1999
- Ponting, Chris P.; Aravind, L.
- Trends in Biochemical Sciences, Vol. 24, Issue 4
Adaptations of the helix-grip fold for ligand binding and catalysis in the START domain superfamily
journal, January 2001
- Iyer, Lakshminarayan M.; Koonin, Eugene V.; Aravind, L.
- Proteins: Structure, Function, and Genetics, Vol. 43, Issue 2
START ships lipids across interorganelle space
journal, January 2014
- Alpy, Fabien; Tomasetto, Catherine
- Biochimie, Vol. 96
Mitochondrial cholesterol: mechanisms of import and effects on mitochondrial function
journal, November 2014
- Martin, Laura A.; Kennedy, Barry E.; Karten, Barbara
- Journal of Bioenergetics and Biomembranes, Vol. 48, Issue 2
Coq10, a mitochondrial coenzyme Q binding protein, is required for proper respiration in Schizosaccharomyces pombe: CoQ-binding protein in fission yeast
journal, December 2008
- Cui, Tie-Zhong; Kawamukai, Makoto
- FEBS Journal, Vol. 276, Issue 3
Oxidoreduction of cytochrome b in the presence of antimycin
journal, December 1972
- Wikström, Mårten K. F.; Berden, Jan A.
- Biochimica et Biophysica Acta (BBA) - Bioenergetics, Vol. 283, Issue 3
Myxothiazol Induces H2O2 Production from Mitochondrial Respiratory Chain
journal, March 2001
- Starkov, Anatoly A.; Fiskum, Gary
- Biochemical and Biophysical Research Communications, Vol. 281, Issue 3
The Mechanism of Mitochondrial Superoxide Production by the Cytochrome bc 1 Complex
journal, June 2008
- Dröse, Stefan; Brandt, Ulrich
- Journal of Biological Chemistry, Vol. 283, Issue 31
Saccharomyces cerevisiae coq10 null mutants are responsive to antimycin A: Coq10p function in coenzyme Q delivery
journal, September 2010
- Busso, Cleverson; Tahara, Erich B.; Ogusucu, Renata
- FEBS Journal, Vol. 277, Issue 21
Identification of the Binding Site of the Quinone-Head Group in Mitochondrial Coq10 by Photoaffinity Labeling
journal, June 2014
- Murai, Masatoshi; Matsunobu, Kohei; Kudo, Sawako
- Biochemistry, Vol. 53, Issue 24
Early Coenzyme Q10 Supplementation in Primary Coenzyme Q10 Deficiency
journal, June 2008
- Montini, Giovanni; Malaventura, Cristina; Salviati, Leonardo
- New England Journal of Medicine, Vol. 358, Issue 26
The Role of Coenzyme Q10 in Statin-Associated Myopathy
journal, June 2007
- Marcoff, Leo; Thompson, Paul D.
- Journal of the American College of Cardiology, Vol. 49, Issue 23
CoQ 10 Function and Role in Heart Failure and Ischemic Heart Disease
journal, July 2015
- Ayer, Anita; Macdonald, Peter; Stocker, Roland
- Annual Review of Nutrition, Vol. 35, Issue 1
The Effect of Coenzyme Q 10 on Morbidity and Mortality in Chronic Heart Failure
journal, December 2014
- Mortensen, Svend A.; Rosenfeldt, Franklin; Kumar, Adarsh
- JACC: Heart Failure, Vol. 2, Issue 6
Characterization of the COQ5 Gene from Saccharomyces cerevisiae EVIDENCE FOR A C -METHYLTRANSFERASE IN UBIQUINONE BIOSYNTHESIS
journal, April 1997
- Barkovich, Robert J.; Shtanko, Andrey; Shepherd, Jennifer A.
- Journal of Biological Chemistry, Vol. 272, Issue 14
Complementation of coq3 Mutant Yeast by Mitochondrial Targeting of the Escherichia coli UbiG Polypeptide: Evidence That UbiG Catalyzes Both O -Methylation Steps in Ubiquinone Biosynthesis †
journal, January 1996
- Hsu, Adam Y.; Poon, Wayne W.; Shepherd, Jennifer A.
- Biochemistry, Vol. 35, Issue 30
Isotope-reinforced polyunsaturated fatty acids protect yeast cells from oxidative stress
journal, January 2011
- Hill, Shauna; Hirano, Kathleen; Shmanai, Vadim V.
- Free Radical Biology and Medicine, Vol. 50, Issue 1
Small amounts of isotope-reinforced polyunsaturated fatty acids suppress lipid autoxidation
journal, August 2012
- Hill, Shauna; Lamberson, Connor R.; Xu, Libin
- Free Radical Biology and Medicine, Vol. 53, Issue 4
Analysis of Molecular Masses and Oligomeric States of Protein Complexes by Blue Native Electrophoresis and Isolation of Membrane Protein Complexes by Two-Dimensional Native Electrophoresis
journal, March 1994
- Schagger, H.; Cramer, W. A.; Vonjagow, G.
- Analytical Biochemistry, Vol. 217, Issue 2
Coenzyme Q supplementation or over-expression of the yeast Coq8 putative kinase stabilizes multi-subunit Coq polypeptide complexes in yeast coq null mutants
journal, April 2014
- He, Cuiwen H.; Xie, Letian X.; Allan, Christopher M.
- Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids, Vol. 1841, Issue 4
Blue native PAGE
journal, June 2006
- Wittig, Ilka; Braun, Hans-Peter; Schägger, Hermann
- Nature Protocols, Vol. 1, Issue 1
para -Aminobenzoic Acid Is a Precursor in Coenzyme Q 6 Biosynthesis in Saccharomyces cerevisiae
journal, June 2010
- Marbois, Beth; Xie, Letian X.; Choi, Samuel
- Journal of Biological Chemistry, Vol. 285, Issue 36
Scalable web services for the PSIPRED Protein Analysis Workbench
journal, June 2013
- Buchan, Daniel W. A.; Minneci, Federico; Nugent, Tim C. O.
- Nucleic Acids Research, Vol. 41, Issue W1
DISOPRED3: precise disordered region predictions with annotated protein-binding activity
journal, November 2014
- Jones, David T.; Cozzetto, Domenico
- Bioinformatics, Vol. 31, Issue 6
SWISS-MODEL: homology modelling of protein structures and complexes
journal, May 2018
- Waterhouse, Andrew; Bertoni, Martino; Bienert, Stefan
- Nucleic Acids Research, Vol. 46, Issue W1
PyRosetta: a script-based interface for implementing molecular modeling algorithms using Rosetta
journal, January 2010
- Chaudhury, S.; Lyskov, S.; Gray, J. J.
- Bioinformatics, Vol. 26, Issue 5, p. 689-691
QMEAN: A comprehensive scoring function for model quality assessment
journal, April 2008
- Benkert, Pascal; Tosatto, Silvio C. E.; Schomburg, Dietmar
- Proteins: Structure, Function, and Bioinformatics, Vol. 71, Issue 1
Assessment of protein models with three-dimensional profiles
journal, March 1992
- Lüthy, Roland; Bowie, James U.; Eisenberg, David
- Nature, Vol. 356, Issue 6364
Verification of protein structures: Patterns of nonbonded atomic interactions
journal, September 1993
- Colovos, Chris; Yeates, Todd O.
- Protein Science, Vol. 2, Issue 9
MolProbity : all-atom structure validation for macromolecular crystallography
journal, December 2009
- Chen, Vincent B.; Arendall, W. Bryan; Headd, Jeffrey J.
- Acta Crystallographica Section D Biological Crystallography, Vol. 66, Issue 1
PHENIX: a comprehensive Python-based system for macromolecular structure solution
journal, January 2010
- Adams, Paul D.; Afonine, Pavel V.; Bunkóczi, Gábor
- Acta Crystallographica Section D Biological Crystallography, Vol. 66, Issue 2, p. 213-221
Enzyme Function Initiative-Enzyme Similarity Tool (EFI-EST): A web tool for generating protein sequence similarity networks
journal, August 2015
- Gerlt, John A.; Bouvier, Jason T.; Davidson, Daniel B.
- Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics, Vol. 1854, Issue 8
Cytoscape: A Software Environment for Integrated Models of Biomolecular Interaction Networks
journal, November 2003
- Shannon, P.
- Genome Research, Vol. 13, Issue 11
UniProt: the universal protein knowledgebase
journal, February 2018
- UniProt Consortium, The
- Nucleic Acids Research, Vol. 46, Issue 5
MAFFT Multiple Sequence Alignment Software Version 7: Improvements in Performance and Usability
journal, January 2013
- Katoh, K.; Standley, D. M.
- Molecular Biology and Evolution, Vol. 30, Issue 4
IQ-TREE: A Fast and Effective Stochastic Algorithm for Estimating Maximum-Likelihood Phylogenies
journal, November 2014
- Nguyen, Lam-Tung; Schmidt, Heiko A.; von Haeseler, Arndt
- Molecular Biology and Evolution, Vol. 32, Issue 1
A RESTful API for Access to Phylogenetic Tools via the CIPRES Science Gateway
journal, January 2015
- Miller, Mark A.; Schwartz, Terri; Pickett, Brett E.
- Evolutionary Bioinformatics, Vol. 11
UFBoot2: Improving the Ultrafast Bootstrap Approximation
journal, October 2017
- Hoang, Diep Thi; Chernomor, Olga; von Haeseler, Arndt
- Molecular Biology and Evolution, Vol. 35, Issue 2
Database resources of the National Center for Biotechnology Information
journal, November 2017
- Agarwala, Richa; Barrett, Tanya; Beck, Jeff
- Nucleic Acids Research, Vol. 46, Issue D1
DMDM: domain mapping of disease mutations
journal, August 2010
- Peterson, T. A.; Adadey, A.; Santana-Cruz, I.
- Bioinformatics, Vol. 26, Issue 19
Statin-induced myopathy: a review and update
journal, February 2011
- Abd, Thura T.; Jacobson, Terry A.
- Expert Opinion on Drug Safety, Vol. 10, Issue 3
Site-directed mutagenesis and structural modeling of Coq10p indicate the presence of a tunnel for coenzyme Q6 binding
journal, March 2010
- Busso, Cleverson; Bleicher, Lucas; Ferreira-Júnior, José Ribamar
- FEBS Letters, Vol. 584, Issue 8
Structural insights into a StART-like domain in Lam4 and its interaction with sterol ligands
journal, January 2018
- Gatta, Alberto T.; Sauerwein, Andrea C.; Zhuravleva, Anastasia
- Biochemical and Biophysical Research Communications, Vol. 495, Issue 3
Molecular basis for sterol transport by St ART ‐like lipid transfer domains
journal, February 2018
- Horenkamp, Florian A.; Valverde, Diana P.; Nunnari, Jodi
- The EMBO Journal, Vol. 37, Issue 6
Structural basis of sterol binding and transport by a yeast StARkin domain
journal, February 2018
- Jentsch, Julian-Alexander; Kiburu, Irene; Pandey, Kalpana
- Journal of Biological Chemistry, Vol. 293, Issue 15
Saccharomyces cerevisiae Coq9 polypeptide is a subunit of the mitochondrial coenzyme Q biosynthetic complex
journal, July 2007
- Hsieh, Edward J.; Gin, Peter; Gulmezian, Melissa
- Archives of Biochemistry and Biophysics, Vol. 463, Issue 1
Ubiquinone biosynthesis in Saccharomyces cerevisiae : the molecular organization of O -methylase Coq3p depends on Abc1p/Coq8p
journal, December 2008
- Tauche, Anja; Krause-Buchholz, Udo; Rödel, Gerhard
- FEMS Yeast Research, Vol. 8, Issue 8
Free Radical Lipid Peroxidation: Mechanisms and Analysis
journal, October 2011
- Yin, Huiyong; Xu, Libin; Porter, Ned A.
- Chemical Reviews, Vol. 111, Issue 10
Mechanisms for the autoxidation of polyunsaturated lipids
journal, September 1986
- Porter, Ned A.
- Accounts of Chemical Research, Vol. 19, Issue 9
Ends of the line for tmRNA-SmpB
journal, August 2014
- Hudson, Corey M.; Lau, Britney Y.; Williams, Kelly P.
- Frontiers in Microbiology, Vol. 5
RatA (YfjG), an Escherichia coli toxin, inhibits 70S ribosome association to block translation initiation: RatA, an inhibitor of 70S ribosome association
journal, February 2011
- Zhang, Yonglong; Inouye, Masayori
- Molecular Microbiology, Vol. 79, Issue 6
SmpB, a unique RNA-binding protein essential for the peptide-tagging activity of SsrA (tmRNA)
journal, July 1999
- Karzai, A. W.
- The EMBO Journal, Vol. 18, Issue 13
Overexpression in Escherichia coli of the rnf genes from Rhodobacter capsulatus . Characterization of two membrane-bound iron-sulfur proteins
journal, January 1998
- Jouanneau, Yves; Jeong, Ho-Sang; Hugo, Nicolas
- European Journal of Biochemistry, Vol. 251, Issue 1-2
Origin and Evolution of the Sodium -Pumping NADH: Ubiquinone Oxidoreductase
journal, May 2014
- Reyes-Prieto, Adrian; Barquera, Blanca; Juárez, Oscar
- PLoS ONE, Vol. 9, Issue 5
Biochemistry, evolution and physiological function of the Rnf complex, a novel ion-motive electron transport complex in prokaryotes
journal, November 2010
- Biegel, Eva; Schmidt, Silke; González, José M.
- Cellular and Molecular Life Sciences, Vol. 68, Issue 4
COXPRESdb in 2015: coexpression database for animal species by DNA-microarray and RNAseq-based expression data with multiple quality assessment systems
journal, November 2014
- Okamura, Yasunobu; Aoki, Yuichi; Obayashi, Takeshi
- Nucleic Acids Research, Vol. 43, Issue D1
The Emerging Network of Mitochondria-Organelle Contacts
journal, March 2016
- Murley, Andrew; Nunnari, Jodi
- Molecular Cell, Vol. 61, Issue 5
An ER-Mitochondria Tethering Complex Revealed by a Synthetic Biology Screen
journal, June 2009
- Kornmann, Benoît; Currie, Erin; Collins, Sean R.
- Science, Vol. 325, Issue 5939
Identification of Coq11, a New Coenzyme Q Biosynthetic Protein in the CoQ-Synthome in Saccharomyces cerevisiae
journal, January 2015
- Allan, Christopher M.; Awad, Agape M.; Johnson, Jarrett S.
- Journal of Biological Chemistry, Vol. 290, Issue 12
Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function
journal, August 2016
- Floyd, Brendan J.; Wilkerson, Emily M.; Veling, Mike T.
- Molecular Cell, Vol. 63, Issue 4
The BioGRID interaction database: 2017 update
journal, December 2016
- Chatr-aryamontri, Andrew; Oughtred, Rose; Boucher, Lorrie
- Nucleic Acids Research, Vol. 45, Issue D1
Targeted exploration and analysis of large cross-platform human transcriptomic compendia
journal, January 2015
- Zhu, Qian; Wong, Aaron K.; Krishnan, Arjun
- Nature Methods, Vol. 12, Issue 3
Polymorphism of Δ3,5-Δ2,4-dienoyl-coenzyme A isomerase (the ECH1 gene product protein) in human striated muscle tissue
journal, April 2006
- Kovalyov, L. I.; Kovalyova, M. A.; Kovalyov, P. L.
- Biochemistry (Moscow), Vol. 71, Issue 4
Tissue-based map of the human proteome
journal, January 2015
- Uhlen, M.; Fagerberg, L.; Hallstrom, B. M.
- Science, Vol. 347, Issue 6220
Analysis of the Human Tissue-specific Expression by Genome-wide Integration of Transcriptomics and Antibody-based Proteomics
journal, December 2013
- Fagerberg, Linn; Hallström, Björn M.; Oksvold, Per
- Molecular & Cellular Proteomics, Vol. 13, Issue 2
Coenzyme Q biosynthetic proteins assemble in a substrate-dependent manner into domains at ER–mitochondria contacts
journal, January 2019
- Subramanian, Kelly; Jochem, Adam; Le Vasseur, Maxence
- The Journal of Cell Biology, Vol. 218, Issue 4
The mammalian START domain protein family in lipid transport in health and disease
journal, September 2011
- Clark, Barbara J.
- Journal of Endocrinology, Vol. 212, Issue 3
MENTHO, a MLN64 Homologue Devoid of the START Domain
journal, October 2002
- Alpy, Fabien; Wendling, Corinne; Rio, Marie-Christine
- Journal of Biological Chemistry, Vol. 277, Issue 52
MLN64 and MENTHO, two mediators of endosomal cholesterol transport
journal, May 2006
- Alpy, F.; Tomasetto, C.
- Biochemical Society Transactions, Vol. 34, Issue 3
Classification and nomenclature of the superfamily of short-chain dehydrogenases/reductases (SDRs)
journal, February 2013
- Persson, Bengt; Kallberg, Yvonne
- Chemico-Biological Interactions, Vol. 202, Issue 1-3
NDUFA9 point mutations cause a variable mitochondrial complex I assembly defect : BAERTLING et al
journal, November 2017
- Baertling, F.; Sánchez-Caballero, L.; van den Brand, M. A. M.
- Clinical Genetics, Vol. 93, Issue 1
Molecular basis of interactions between mitochondrial proteins and hydroxyapatite in the presence of Triton X-100, as revealed by proteomic and recombinant techniques
journal, August 2013
- Yamamoto, Takenori; Tamaki, Haruna; Katsuda, Chie
- Journal of Chromatography A, Vol. 1301
Biotin and Lipoic Acid: Synthesis, Attachment, and Regulation
journal, December 2013
- Cronan, John E.
- EcoSal Plus, Vol. 1, Issue 3
Mitochondrial Fatty Acid Synthesis Type II: More than Just Fatty Acids
journal, November 2008
- Hiltunen, J. Kalervo; Schonauer, Melissa S.; Autio, Kaija J.
- Journal of Biological Chemistry, Vol. 284, Issue 14
Exploring the functional landscape of gene expression: directed search of large microarray compendia
journal, August 2007
- Hibbs, M. A.; Hess, D. C.; Myers, C. L.
- Bioinformatics, Vol. 23, Issue 20
Peroxisomes are juxtaposed to strategic sites on mitochondria
journal, January 2014
- Cohen, Yifat; Klug, Yoel Alexander; Dimitrov, Lazar
- Mol. BioSyst., Vol. 10, Issue 7
Mitofusin 2 Builds a Bridge between ER and Mitochondria
journal, December 2008
- Merkwirth, Carsten; Langer, Thomas
- Cell, Vol. 135, Issue 7
Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels
journal, February 2015
- Mourier, Arnaud; Motori, Elisa; Brandt, Tobias
- The Journal of Cell Biology, Vol. 208, Issue 4
Genetic Evidence for a Multi-subunit Complex in Coenzyme Q Biosynthesis in Yeast and the Role of the Coq1 Hexaprenyl Diphosphate Synthase
journal, November 2004
- Gin, Peter; Clarke, Catherine F.
- Journal of Biological Chemistry, Vol. 280, Issue 4
Enhanced sensitivity of ubiquinone-deficient mutants of Saccharomyces cerevisiae to products of autoxidized polyunsaturated fatty acids.
journal, July 1996
- Do, T. Q.; Schultz, J. R.; Clarke, C. F.
- Proceedings of the National Academy of Sciences, Vol. 93, Issue 15
Genetic evidence for a multi-subunit complex in the O-methyltransferase steps of coenzyme Q biosynthesis
journal, April 2000
- Hsu, Adam Y.; Do, Thai Q.; Lee, Peter T.
- Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids, Vol. 1484, Issue 2-3
The Saccharomyces cerevisiae COQ6 Gene Encodes a Mitochondrial Flavin-dependent Monooxygenase Required for Coenzyme Q Biosynthesis
journal, April 2003
- Gin, Peter; Hsu, Adam Y.; Rothman, Steven C.
- Journal of Biological Chemistry, Vol. 278, Issue 28
The COQ7 Gene Encodes a Protein in Saccharomyces cerevisiae Necessary for Ubiquinone Biosynthesis
journal, February 1996
- Marbois, B. Noelle; Clarke, Catherine F.
- Journal of Biological Chemistry, Vol. 271, Issue 6
COQ9 , a New Gene Required for the Biosynthesis of Coenzyme Q in Saccharomyces cerevisiae
journal, July 2005
- Johnson, Alisha; Gin, Peter; Marbois, Beth N.
- Journal of Biological Chemistry, Vol. 280, Issue 36
Yeast and Rat Coq3 and Escherichia coli UbiG Polypeptides Catalyze Both O -Methyltransferase Steps in Coenzyme Q Biosynthesis
journal, July 1999
- Poon, Wayne W.; Barkovich, Robert J.; Hsu, Adam Y.
- Journal of Biological Chemistry, Vol. 274, Issue 31
Yeast COQ4 Encodes a Mitochondrial Protein Required for Coenzyme Q Synthesis
journal, August 2001
- Belogrudov, Grigory I.; Lee, Peter T.; Jonassen, Tanya
- Archives of Biochemistry and Biophysics, Vol. 392, Issue 1
Yeast Coq5 C -Methyltransferase Is Required for Stability of Other Polypeptides Involved in Coenzyme Q Biosynthesis
journal, December 2003
- Baba, Suzie W.; Belogrudov, Grigory I.; Lee, Justine C.
- Journal of Biological Chemistry, Vol. 279, Issue 11
Complementation of Saccharomyces cerevisiae coq7 Mutants by Mitochondrial Targeting of the Escherichia coli UbiF Polypeptide : TWO FUNCTIONS OF YEAST COQ7 POLYPEPTIDE IN COENZYME Q BIOSYNTHESIS
journal, April 2006
- Tran, UyenPhuong C.; Marbois, Beth; Gin, Peter
- Journal of Biological Chemistry, Vol. 281, Issue 24
A tRNATRP gene mediates the suppression of cbs2-223 previously attributed to ABC1/COQ8
journal, April 2004
- Hsieh, Edward J.; Dinoso, Jason B.; Clarke, Catherine F.
- Biochemical and Biophysical Research Communications, Vol. 317, Issue 2
Works referencing / citing this record:
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