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
ubiI, a New Gene in Escherichia coli Coenzyme Q Biosynthesis, Is Involved in Aerobic C5-hydroxylation *
Mahmoud Hajj Chehade,Mahmoud Hajj Chehade,Mahmoud Hajj Chehade,Laurent Loiseau,Murielle Lombard,Ludovic Pecqueur,Alexandre Ismail,Myriam Smadja,Béatrice Golinelli-Pimpaneau,Caroline Mellot-Draznieks,Olivier Hamelin,Olivier Hamelin,Olivier Hamelin,Laurent Aussel,Sylvie Kieffer-Jaquinod,Natty Labessan,Frédéric Barras,Marc Fontecave,Fabien Pierrel,Fabien Pierrel,Fabien Pierrel +20 more
TL;DR: This work shows that the uncharacterized conserved visC gene is involved in Q biosynthesis in Escherichia coli, and it is renamed ubiI, and the crystal structure of a truncated form of UbiI is solved, the first structural characterization of a monooxygenase involved inQ biosynthesis.
Journal ArticleDOI
A novel mutation in COQ2 leading to fatal infantile multisystem disease
Bernadette S. Jakobs,Lambert P. van den Heuvel,Roel J.P. Smeets,Maaike de Vries,Steffen Hien,Thomas Schaible,Jan A.M. Smeitink,Ron A. Wevers,Saskia B. Wortmann,Richard J. Rodenburg +9 more
TL;DR: A novel homozygous mutation in COQ2 is reported in a dizygotic twin from consanguineous Turkish parents with primary coenzyme Q10 deficiency, which could be under diagnosed in patients with fatal neonatal or infantile multi-organ disease.
Journal ArticleDOI
Focal segmental glomerulosclerosis is associated with a PDSS2 haplotype and, independently, with a decreased content of coenzyme Q10
David L. Gasser,Cheryl A. Winkler,Min Peng,Ping An,Louise A. McKenzie,Gregory D. Kirk,Yuchen Shi,Letian X. Xie,Beth N. Marbois,Catherine F. Clarke,Jeffrey B. Kopp +10 more
TL;DR: It is suggested that FSGS patients have Q10 deficiency and that this deficiency is manifested in patient-derived lymphoblastoid cell lines from FS GS patients, which is independent of PDSS2 haplotype.
Journal ArticleDOI
Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis.
Theresa Nguyen,Alberto Casarin,Maria Andrea Desbats,Mara Doimo,Eva Trevisson,Carlos Santos-Ocaña,Plácido Navas,Catherine F. Clarke,Leonardo Salviati +8 more
TL;DR: The results presented suggest that human and Escherichia coli Coq5 homologs expressed in yeast retain C-methyltransferase activity but are capable of rescuing the coq5 yeast mutants only when the CoQ-synthome is assembled.
Journal ArticleDOI
Cardiofaciocutaneous (CFC) syndrome associated with muscular coenzyme Q10 deficiency.
TL;DR: In this paper, the authors reported the case of a 4-year-old girl who presented a CFC syndrome, confirmed by the presence of a pathogenic R257Q BRAF gene mutation, together with a muscular CoQ10 deficiency.
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