MT-CO1 mRNA is translated

Stable Identifier
R-HSA-9865309
Type
Reaction [uncertain]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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Mitoribosomes translate MT-CO1 mRNA, after which the nascent protein localizes to the inner mitochondrial membrane. Translation of MT-CO1 requires Translational activator of cytochrome c oxidase 1 (TACO1, CCDC44) bound to a complex consisting of MR-1S, PET100, and PET117 (Weraarpachai et al., 2009; Vidoni et al., 2017; Sun et al., 2023). Another positive regulator appears to be TMEM227 (Dennerlein et al., 2021). Also, the process is enhanced by COX4 as part of a later stage of the assembly complex (Richter-Dennerlein et al., 2016).
Literature References
PubMed ID Title Journal Year
34969438 Defining the interactome of the human mitochondrial ribosome identifies SMIM4 and TMEM223 as respiratory chain assembly factors

Hanitsch, E, Bauermeister, D, Wang, C, Stoldt, S, Poerschke, S, Jakobs, S, Langer, T, Dennerlein, S, Sattmann, J, Richter-Dennerlein, R, Warscheid, B, Rehling, P, Oeljeklaus, S

Elife 2021
27693358 Mitochondrial Protein Synthesis Adapts to Influx of Nuclear-Encoded Protein

Lorenzi, I, Schendzielorz, AB, Wang, C, Richter-Dennerlein, R, Warscheid, B, Bareth, B, Rehling, P, Ronsör, C, Oeljeklaus, S, Dennerlein, S

Cell 2016
37247699 PET117 assembly factor stabilizes translation activator TACO1 thereby upregulates mitochondria-encoded cytochrome C oxidase 1 synthesis

Liu, J, Shi, L, Long, J, Liu, H, Li, J, Yang, Z, Huang, X, Sun, Q, Zhang, R, Peng, Y, Li, S, Shao, Y, Feng, Z

Free Radic Biol Med 2023
19503089 Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome

Schrank, B, Lochmüller, H, Antonicka, H, Seeger, J, Chevrette, M, Horvath, R, Sasarman, F, Shoubridge, EA, Kolesar, JE, Weraarpachai, W, Kaufman, BA

Nat Genet 2009
28199844 MR-1S Interacts with PET100 and PET117 in Module-Based Assembly of Human Cytochrome c Oxidase

Signes, A, Vidoni, S, Taylor, RW, Fearnley, IM, Harbour, ME, Arnold, S, Guerrero-Castillo, S, Fernandez-Vizarra, E, Ding, S, Zeviani, M, Tiranti, V

Cell Rep 2017
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