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Structure and functionality of a multimeric human COQ7:COQ9 complex.

Manicki M, Aydin H, Abriata LA, Overmyer KA, Guerra RM, Coon JJ, Dal Peraro M, Frost A, Pagliarini DJ
Mol Cell (2022)
Cited: 14 times
EuropePMC logo PMID: 36306796

Abstract

Coenzyme Q (CoQ) is a redox-active lipid essential for core metabolic pathways and antioxidant defense. CoQ is synthesized upon the mitochondrial inner membrane by an ill-defined "complex Q" metabolon. Here, we present structure-function analyses of a lipid-, substrate-, and NADH-bound complex comprising two complex Q subunits: the hydroxylase COQ7 and the lipid-binding protein COQ9. We reveal that COQ7 adopts a ferritin-like fold with a hydrophobic channel whose substrate-binding capacity is enhanced by COQ9. Using molecular dynamics, we further show that two COQ7:COQ9 heterodimers form a curved tetramer that deforms the membrane, potentially opening a pathway for the CoQ intermediates to translocate from the bilayer to the proteins' lipid-binding sites. Two such tetramers assemble into a soluble octamer with a pseudo-bilayer of lipids captured within. Together, these observations indicate that COQ7 and COQ9 cooperate to access hydrophobic precursors within the membrane and coordinate subsequent synthesis steps toward producing CoQ.

Articles - 7ssp mentioned but not cited (4)

  1. Structure and functionality of a multimeric human COQ7:COQ9 complex. Manicki M, Aydin H, Abriata LA, Overmyer KA, Guerra RM, Coon JJ, Dal Peraro M, Frost A, Pagliarini DJ. Mol Cell 82 4307-4323.e10 (2022)
  2. Predicting and Understanding the Pathology of Single Nucleotide Variants in Human COQ Genes. Wang S, Jain A, Novales NA, Nashner AN, Tran F, Clarke CF. Antioxidants (Basel) 11 2308 (2022)
  3. Homozygous variant in COQ7 causes autosomal recessive hereditary spastic paraplegia. Qiu Y, Xiong Y, Wang L, Zhu M, Tan D, Hong D. Ann Clin Transl Neurol 11 1067-1074 (2024)
  4. Identification of novel coenzyme Q10 biosynthetic proteins Coq11 and Coq12 in Schizosaccharomyces pombe. Nishida I, Ohmori Y, Yanai R, Nishihara S, Matsuo Y, Kaino T, Hirata D, Kawamukai M. J Biol Chem 299 104797 (2023)


Reviews citing this publication (3)

  1. Coenzyme Q biochemistry and biosynthesis. Guerra RM, Pagliarini DJ. Trends Biochem Sci 48 463-476 (2023)
  2. Biosynthesis, Deficiency, and Supplementation of Coenzyme Q. Staiano C, García-Corzo L, Mantle D, Turton N, Millichap LE, Brea-Calvo G, Hargreaves I. Antioxidants (Basel) 12 1469 (2023)
  3. Mitochondrial Factors in the Cell Nucleus. González-Arzola K, Díaz-Quintana A. Int J Mol Sci 24 13656 (2023)

Articles citing this publication (7)

  1. Structural mechanism of mitochondrial membrane remodelling by human OPA1. von der Malsburg A, Sapp GM, Zuccaro KE, von Appen A, Moss FR, Kalia R, Bennett JA, Abriata LA, Dal Peraro M, van der Laan M, Frost A, Aydin H. Nature 620 1101-1108 (2023)
  2. Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy. Jacquier A, Theuriet J, Fontaine F, Mosbach V, Lacoste N, Ribault S, Risson V, Carras J, Coudert L, Simonet T, Latour P, Stojkovic T, Piard J, Cosson A, Lesca G, Bouhour F, Allouche S, Puccio H, Pegat A, Schaeffer L. Brain 146 3470-3483 (2023)
  3. Phenotypic, molecular, and functional characterization of COQ7-related primary CoQ10 deficiency: Hypomorphic variants and two distinct disease entities. Wongkittichote P, Duque Lasio ML, Magistrati M, Pathak S, Sample B, Carvalho DR, Ortega AB, Castro MAA, de Gusmao CM, Toler TL, Bellacchio E, Dallabona C, Shinawi M. Mol Genet Metab 139 107630 (2023)
  4. The structure of the human LACTB filament reveals the mechanisms of assembly and membrane binding. Bennett JA, Steward LR, Rudolph J, Voss AP, Aydin H. PLoS Biol 20 e3001899 (2022)
  5. An ETFDH-driven metabolon supports OXPHOS efficiency in skeletal muscle by regulating coenzyme Q homeostasis. Herrero Martín JC, Salegi Ansa B, Álvarez-Rivera G, Domínguez-Zorita S, Rodríguez-Pombo P, Pérez B, Calvo E, Paradela A, Miguez DG, Cifuentes A, Cuezva JM, Formentini L. Nat Metab 6 209-225 (2024)
  6. COQ7 defect causes prenatal onset of mitochondrial CoQ10 deficiency with cardiomyopathy and gastrointestinal obstruction. Pettenuzzo I, Carli S, Sánchez-Cuesta A, Isidori F, Montanari F, Grippa M, Lanzoni G, Ambrosetti I, Di Pisa V, Cordelli DM, Mondardini MC, Pippucci T, Ragni L, Cenacchi G, Costa R, Lima M, Capristo MA, Tropeano CV, Caporali L, Carelli V, Brunelli E, Maffei M, Ahmed Sheikhmaye H, Fetta A, Brea-Calvo G, Garone C. Eur J Hum Genet (2024)
  7. Characterization of Arabidopsis thaliana Coq9 in the CoQ Biosynthetic Pathway. Hu M, Jiang Y, Xu JJ. Metabolites 13 813 (2023)


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