Biochemistry of Mitochondrial Coenzyme Q Biosynthesis.
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TLDR
This work highlights recent progress toward filling knowledge gaps in CoQ biosynthesis through both traditional biochemistry and cutting-edge 'omics' approaches and presents questions framed by the recently discovered Co Q biosynthesis complex and by putative biophysical barriers.About:
This article is published in Trends in Biochemical Sciences.The article was published on 2017-10-01 and is currently open access. It has received 197 citations till now.read more
Citations
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Proteomic Mapping of Mitochondria in Living Cells via Spatially Restricted Enzymatic Tagging
Hyun-Woo Rhee,Peng Zou,Namrata D. Udeshi,Jeffrey D. Martell,Vamsi K. Mootha,Vamsi K. Mootha,Steven A. Carr,Alice Y. Ting,Alice Y. Ting +8 more
TL;DR: In this article, a peroxidase-based method was used to identify 495 proteins within the human mitochondrial matrix, including 31 proteins not previously linked to mitochondria, and the labeling was exceptionally specific and distinguished between inner membrane proteins facing the matrix versus the intermembrane space.
Journal ArticleDOI
Targeting ferroptosis as a vulnerability in cancer
Guang Lei,Li Zhuang,Boyi Gan +2 more
TL;DR: The current understanding of ferroptosis-inducing and ferroPTosis defence mechanisms is summarized, the roles and mechanisms of ferraptosis in tumour suppression and tumour immunity are dissected, and therapeutic strategies for targeting ferroaptosis in cancer are explored.
Journal ArticleDOI
Mitochondrial dysfunction-related lipid changes occur in nonalcoholic fatty liver disease progression
Kang-Yu Peng,Kang-Yu Peng,Matthew J. Watt,Sander S. Rensen,Jan Greve,Kevin Huynh,Kaushala S. Jayawardana,Peter J. Meikle,Peter J. Meikle,Ruth C. R. Meex +9 more
TL;DR: It is proposed that increased levels of cardiolipin and ubiquinone may help to preserve mitochondrial function in early NAFLD, but that mitochondrial function eventually fails with progression to NASH, leading to increased acylcarnitine.
Journal ArticleDOI
Mitochondrial regulation of ferroptosis.
TL;DR: In this paper, the authors summarize the diverse metabolic processes in mitochondria that actively drive ferroptosis, discuss recently discovered mitochondria-localized defense systems that detoxify mitochondrial lipid peroxides and protect against ferrosinosis, and outline outstanding questions on this fascinating topic for future investigations.
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Coenzyme Q10 deficiencies: pathways in yeast and humans.
Agape M. Awad,Michelle C. Bradley,Lucía Fernández-del-Río,Anish Nag,Hui S. Tsui,Catherine F. Clarke +5 more
TL;DR: Yeast provides a simple yet effective model to investigate and define the function and possible pathology of human COQ (yeast or human gene involved in CoQ biosynthesis) gene polymorphisms and mutations.
References
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Journal ArticleDOI
Crystal structures and catalytic mechanism of the C-methyltransferase Coq5 provide insights into a key step of the yeast coenzyme Q synthesis pathway.
Ya-Nan Dai,Kang Zhou,Dong-Dong Cao,Yong-Liang Jiang,Fei Meng,Chang-Biao Chi,Yan-Min Ren,Yuxing Chen,Cong-Zhao Zhou +8 more
TL;DR: Structural-based computational simulation using an analogue of DDMQH2 enabled us to identify the binding pocket and entrance tunnel of the substrate and multiple-sequence alignment showed that the residues contributing to the dimeric interface and the SAM- and DDMQh2-binding sites are highly conserved in Coq5 and homologues from diverse species.
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2-multiprenylphenols and 2-decaprenyl-6-methoxyphenol, biosynthetic precursors of ubiquinones.
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Aging-Associated Enzyme Human Clock-1: Substrate-Mediated Reduction of the Diiron Center for 5-Demethoxyubiquinone Hydroxylation
TL;DR: In this article, a human Clock-1 (hCLK-1) fusion protein with an N-terminal immunoglobulin binding domain of protein G (GB1) was expressed and characterized by spectroscopic and kinetic methods.
Journal ArticleDOI
Identification of 3-methoxy-4-hydroxy-5-hexaprenylbenzoic acid as a new intermediate in ubiquinone biosynthesis by Saccharomyces cerevisiae.
TL;DR: It is found that 3-O-methylation precedes decarboxylation of the prenylated protocatechuic acid intermediate in the biosynthesis of ubiquinone in eukaryotes and in vitro experiments with isolated yeast and rat mitochondria showed that3-MHHB could be converted to ubiquin one-6.
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Disruption of the human COQ5-containing protein complex is associated with diminished coenzyme Q10 levels under two different conditions of mitochondrial energy deficiency
Hsiu-Chuan Yen,Hsiu-Chuan Yen,Yi-Chun Liu,Chia-Chi Kan,Hsing-Ju Wei,Szu-Hsien Lee,Yau-Huei Wei,Yu-Hsiu Feng,Chih-Wei Chen,Chin-Chang Huang +9 more
TL;DR: The findings elucidate a possible mechanism for mitochondrial dysfunction-induced CoQ10 deficiency in human cells and discovery of a novel COQ5-containing protein complex was discovered inhuman cells.
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