Coiled-coil-helix-coiled-coil-helix domain-containing protein 1 / Cysteine alpha-hairpin motif superfamily / 28S ribosomal protein S26 / Mitochondrial ribosome subunit S26 / : / 28S ribosomal protein S27, mitochondrial / Ribosomal protein S23, mitochondrial / Ribosomal protein S23/S25, mitochondrial / Mitochondrial 28S ribosomal protein S31 / MRPS27/PTCD2 ...Coiled-coil-helix-coiled-coil-helix domain-containing protein 1 / Cysteine alpha-hairpin motif superfamily / 28S ribosomal protein S26 / Mitochondrial ribosome subunit S26 / : / 28S ribosomal protein S27, mitochondrial / Ribosomal protein S23, mitochondrial / Ribosomal protein S23/S25, mitochondrial / Mitochondrial 28S ribosomal protein S31 / MRPS27/PTCD2 / Mitochondrial 28S ribosomal protein S27 / Mitochondrial ribosomal protein S23 / Mitochondrial 28S ribosomal protein S31 / Ribosomal protein S22, mitochondrial / : / Mitochondrial 28S ribosomal protein S22 / Ribosomal protein S29, mitochondrial / Ribosomal protein S28, mitochondrial / 28S ribosomal protein S18b, mitochondrial / 28S ribosomal protein S10, mitochondrial / Mitochondrial ribosomal protein MRP-S35 / 28S ribosomal protein S24, mitochondrial / Mitochondrial 28S ribosomal protein S34 / Pentatricopeptide repeat domain-containing protein 3 / 28S ribosomal protein S17, mitochondrial / : / : / Mitochondrial ribosome subunit S24 / Mitochondrial 28S ribosomal protein S34 / Small ribosomal subunit protein uS5m, N-terminal / Small ribosomal subunit protein mS39, PPR / 28S ribosomal protein S25, mitochondrial / : / Ribosomal protein L55, mitochondrial / Mitochondrial ribosomal protein L55 superfamily / : / Mitochondrial ribosomal protein L55 / Mitochondrial ribosomal protein L48 / 39S ribosomal protein L40, mitochondrial / Pentatricopeptide repeat domain / Ribosomal protein S30, mitochondrial / Ribosomal protein L53, mitochondrial / 39S ribosomal protein L53/MRP-L53 / 39S ribosomal protein L42, mitochondrial / Mitochondrial 28S ribosomal protein S32 / Ribosomal protein L37, mitochondrial / Mitochondrial ribosomal protein L37 / Ribosomal protein 63, mitochondrial / Growth arrest/ DNA-damage-inducible protein-interacting protein 1 / Ribosomal protein L35, mitochondrial / Ribosomal protein L51, mitochondrial / Growth arrest and DNA-damage-inducible proteins-interacting protein 1 / Mitochondrial ribosomal subunit / Mitochondrial ribosome protein 63 / Ribosomal protein L37/S30 / Ribosomal protein S27/S33, mitochondrial / Ribosomal protein S24/S35, mitochondrial / Growth arrest and DNA damage-inducible proteins-interacting protein 1 domain superfamily / : / : / Mitochondrial 28S ribosomal protein S30 (PDCD9) / Mitochondrial ribosomal subunit S27 / 39S ribosomal protein L52, mitochondrial / Mitoribosomal protein mL52 / : / : / Large ribosomal subunit protein mL44, endonuclease domain / Ribosomal protein L28/L40, mitochondrial / Mitochondrial ribosomal protein L28 / Ribosomal protein S24/S35, mitochondrial, conserved domain / : / : / Mitochondrial ribosomal subunit protein / Large ribosomal subunit protein bL9m C-terminal domain / Large ribosomal subunit protein bL9m N-terminal domain / Tim44-like domain / Tim44-like domain / Tim44 / : / Pentatricopeptide (PPR) repeat profile. / Ribosomal protein L7/L12, oligomerisation / Ribosomal protein L7/L12, oligomerisation domain superfamily / Ribosomal protein L7/L12 dimerisation domain / : / Ribosomal protein L7/L12 / Ribosomal protein L7/L12, C-terminal / MRPL44, double-stranded RNA binding domain / Ribosomal protein L7/L12 C-terminal domain / MRPL44 dsRNA-binding domain / Mitochondrial ribosomal protein L46 NUDIX / Ribosomal protein L50, mitochondria / Ribosomal subunit 39S / Pentatricopeptide repeat / Ribosomal protein L46, N-terminal / 39S mitochondrial ribosomal protein L46 / Ribosomal protein S23/S29, mitochondrial / Mitochondrial ribosomal death-associated protein 3 / Ribosomal protein L49/IMG2 / Mitochondrial large subunit ribosomal protein (Img2) / Ribosomal protein L27/L41, mitochondrial Similarity search - Domain/homology
ADENOSINE-5'-TRIPHOSPHATE / FE2/S2 (INORGANIC) CLUSTER / Retapamulin / GUANOSINE-5'-DIPHOSPHATE / : / NICOTINAMIDE-ADENINE-DINUCLEOTIDE / SPERMIDINE / VALINE / : / : ...ADENOSINE-5'-TRIPHOSPHATE / FE2/S2 (INORGANIC) CLUSTER / Retapamulin / GUANOSINE-5'-DIPHOSPHATE / : / NICOTINAMIDE-ADENINE-DINUCLEOTIDE / SPERMIDINE / VALINE / : / : / : / RNA / RNA (> 10) / RNA (> 100) / RNA (> 1000) / MRPS21 isoform 1 / Small ribosomal subunit protein uS12m / Small ribosomal subunit protein uS14m / Large ribosomal subunit protein bL33m / Large ribosomal subunit protein uL3m / Large ribosomal subunit protein bL19m / Small ribosomal subunit protein mS29 / Large ribosomal subunit protein bL12m / Small ribosomal subunit protein mS22 / Small ribosomal subunit protein mS25 / Small ribosomal subunit protein uS10m / Small ribosomal subunit protein mS35 / Small ribosomal subunit protein uS5m / Small ribosomal subunit protein uS11m / Small ribosomal subunit protein uS15m / Small ribosomal subunit protein mS34 / Small ribosomal subunit protein bS6m / Small ribosomal subunit protein uS9m / Large ribosomal subunit protein bL28m / Large ribosomal subunit protein mL49 / Large ribosomal subunit protein mL62 / Large ribosomal subunit protein uL23m / Large ribosomal subunit protein mL51 / Large ribosomal subunit protein uL2m / Large ribosomal subunit protein mL54 / Large ribosomal subunit protein uL14m / Large ribosomal subunit protein bL21m / Large ribosomal subunit protein mL55 / Large ribosomal subunit protein uL10m / Large ribosomal subunit protein mL52 / Large ribosomal subunit protein mL41 / Large ribosomal subunit protein mL50 / Large ribosomal subunit protein mL43 / Large ribosomal subunit protein mL64 / Large ribosomal subunit protein uL30m / Small ribosomal subunit protein mS27 / Small ribosomal subunit protein mS31 / Large ribosomal subunit protein uL24m / Small ribosomal subunit protein mS37 / Large ribosomal subunit protein mL38 / Small ribosomal subunit protein uS3m / Large ribosomal subunit protein mL53 / Small ribosomal subunit protein mS39 / Large ribosomal subunit protein mL48 / Large ribosomal subunit protein bL34m / Large ribosomal subunit protein mL63 / Large ribosomal subunit protein mL45 / Large ribosomal subunit protein bL32m / Large ribosomal subunit protein bL20m / Large ribosomal subunit protein uL13m / Large ribosomal subunit protein bL9m / Large ribosomal subunit protein uL4m / Small ribosomal subunit protein mS26 / Large ribosomal subunit protein mL37 / Large ribosomal subunit protein uL18m / Large ribosomal subunit protein mL46 / Large ribosomal subunit protein mL44 / Large ribosomal subunit protein uL29m / Large ribosomal subunit protein mL65 / Large ribosomal subunit protein mL40 / Large ribosomal subunit protein bL17m / Large ribosomal subunit protein mL66 / Small ribosomal subunit protein bS22, mitochondrial / Large ribosomal subunit protein uL22m / Large ribosomal subunit protein uL16m / Large ribosomal subunit protein mL39 / Large ribosomal subunit protein bL35m / Large ribosomal subunit protein uL15m / Large ribosomal subunit protein bL36m / Large ribosomal subunit protein bL27m / Small ribosomal subunit protein mS33 / Small ribosomal subunit protein bS1m / Small ribosomal subunit protein uS17m / Small ribosomal subunit protein uS7m / Small ribosomal subunit protein uS2m / Large ribosomal subunit protein uL11m / Small ribosomal subunit protein bS16m / Small ribosomal subunit protein bS18m / Small ribosomal subunit protein mS23 / Small ribosomal subunit protein mS40 / Large ribosomal subunit protein mL42 Similarity search - Component
Biological species
Homo sapiens (human)
Method
ELECTRON MICROSCOPY / single particle reconstruction / cryo EM / Resolution: 3 Å
Journal: Mol Cell / Year: 2025 Title: Monitoring the complexity and dynamics of mitochondrial translation. Authors: Taisei Wakigawa / Mari Mito / Yushin Ando / Haruna Yamashiro / Kotaro Tomuro / Haruna Tani / Kazuhito Tomizawa / Takeshi Chujo / Asuteka Nagao / Takeo Suzuki / Osamu Nureki / Fan-Yan Wei / ...Authors: Taisei Wakigawa / Mari Mito / Yushin Ando / Haruna Yamashiro / Kotaro Tomuro / Haruna Tani / Kazuhito Tomizawa / Takeshi Chujo / Asuteka Nagao / Takeo Suzuki / Osamu Nureki / Fan-Yan Wei / Yuichi Shichino / Yuzuru Itoh / Tsutomu Suzuki / Shintaro Iwasaki / Abstract: Since mitochondrial translation leads to the synthesis of the essential oxidative phosphorylation (OXPHOS) subunits, exhaustive and quantitative delineation of mitoribosome traversal is needed. Here, ...Since mitochondrial translation leads to the synthesis of the essential oxidative phosphorylation (OXPHOS) subunits, exhaustive and quantitative delineation of mitoribosome traversal is needed. Here, we developed a variety of high-resolution mitochondrial ribosome profiling derivatives and revealed the intricate regulation of mammalian mitochondrial translation. Harnessing a translation inhibitor, retapamulin, our approach assessed the stoichiometry and kinetics of mitochondrial translation flux, such as the number of mitoribosomes on a transcript, the elongation rate, and the initiation rate. We also surveyed the impacts of modifications at the anticodon stem loop in mitochondrial tRNAs (mt-tRNAs), including all possible modifications at the 34th position, in cells deleting the corresponding enzymes and derived from patients, as well as in mouse tissues. Moreover, a retapamulin-assisted derivative and mito-disome profiling revealed mitochondrial translation initiation factor (mtIF) 3-mediated translation initiation from internal open reading frames (ORFs) and programmed mitoribosome collision sites across the mitochondrial transcriptome. Our work provides a useful platform for investigating protein synthesis within the energy powerhouse of the cell.
A: 16S mitochondrial rRNA B: mitochondrial tRNAVal D: Large ribosomal subunit protein uL2m E: Large ribosomal subunit protein uL3m F: Large ribosomal subunit protein uL4m H: Large ribosomal subunit protein bL9m I: Large ribosomal subunit protein uL10m J: Large ribosomal subunit protein uL11m K: Large ribosomal subunit protein uL13m L: Large ribosomal subunit protein uL14m M: Large ribosomal subunit protein uL15m N: Large ribosomal subunit protein uL16m O: Large ribosomal subunit protein bL17m P: Large ribosomal subunit protein uL18m Q: Large ribosomal subunit protein bL19m R: Large ribosomal subunit protein bL20m S: Large ribosomal subunit protein bL21m T: Large ribosomal subunit protein uL22m U: Large ribosomal subunit protein uL23m V: Large ribosomal subunit protein uL24m W: Large ribosomal subunit protein bL27m X: Large ribosomal subunit protein bL28m Y: Large ribosomal subunit protein uL29m Z: Large ribosomal subunit protein uL30m AA: 12S mitochondrial rRNA AB: Small ribosomal subunit protein uS2m AC: Small ribosomal subunit protein uS3m AD: Small ribosomal subunit protein uS5m AE: Small ribosomal subunit protein bS6m AF: Small ribosomal subunit protein uS7m AG: Small ribosomal subunit protein uS9m AH: Small ribosomal subunit protein uS10m AI: Small ribosomal subunit protein uS11m AJ: Small ribosomal subunit protein uS12m AK: Small ribosomal subunit protein uS14m AL: Small ribosomal subunit protein uS15m AM: Small ribosomal subunit protein bS16m AN: Small ribosomal subunit protein uS17m AO: Small ribosomal subunit protein mS40 AP: Small ribosomal subunit protein bS18m AQ: MRPS21 isoform 1 AR: Small ribosomal subunit protein mS22 AS: Small ribosomal subunit protein mS23 AT: Small ribosomal subunit protein mS25 AU: Small ribosomal subunit protein mS26 AV: Small ribosomal subunit protein mS27 AW: Small ribosomal subunit protein bS1m AX: Small ribosomal subunit protein mS29 AY: Small ribosomal subunit protein mS31 AZ: Small ribosomal subunit protein mS33 A0: Small ribosomal subunit protein mS34 A1: Small ribosomal subunit protein mS35 A2: Small ribosomal subunit protein mS37 A3: Small ribosomal subunit protein mS38 A4: Small ribosomal subunit protein mS39 Ax: P/P-tRNA Az: mRNA 0: Large ribosomal subunit protein bL32m 1: Large ribosomal subunit protein bL33m 2: Large ribosomal subunit protein bL34m 3: Large ribosomal subunit protein bL35m 4: Large ribosomal subunit protein bL36m 5: Large ribosomal subunit protein mL37 6: Large ribosomal subunit protein mL38 7: Large ribosomal subunit protein mL39 8: Large ribosomal subunit protein mL40 9: Large ribosomal subunit protein mL41 a: Large ribosomal subunit protein mL42 b: Large ribosomal subunit protein mL43 c: Large ribosomal subunit protein mL44 d: Large ribosomal subunit protein mL45 e: Large ribosomal subunit protein mL46 f: Large ribosomal subunit protein mL48 g: Large ribosomal subunit protein mL49 h: Large ribosomal subunit protein mL50 i: Large ribosomal subunit protein mL51 j: Large ribosomal subunit protein mL52 k: Large ribosomal subunit protein mL53 l: Large ribosomal subunit protein mL54 m: Large ribosomal subunit protein mL55 o: Large ribosomal subunit protein mL63 p: Large ribosomal subunit protein mL62 q: Large ribosomal subunit protein mL64 r: Large ribosomal subunit protein mL66 s: Large ribosomal subunit protein mL65 t: Large ribosomal subunit protein bL12m hetero molecules
Mass: 23674.203 Da / Num. of mol.: 1 / Source method: isolated from a natural source / Source: (natural) Homo sapiens (human) / References: UniProt: Q14197, peptidyl-tRNA hydrolase
#83: Protein
LargeribosomalsubunitproteinmL64 / 39S ribosomal protein L59 / mitochondrial / MRP-L59 / CKII beta-associating protein / CR6- ...39S ribosomal protein L59 / mitochondrial / MRP-L59 / CKII beta-associating protein / CR6-interacting factor 1 / CRIF1 / Growth arrest and DNA damage-inducible proteins-interacting protein 1 / Papillomavirus L2-interacting nuclear protein 1 / PLINP / PLINP-1 / p53-responsive gene 6 protein
Mass: 25426.895 Da / Num. of mol.: 1 / Source method: isolated from a natural source / Source: (natural) Homo sapiens (human) / References: UniProt: Q8TAE8
#84: Protein
LargeribosomalsubunitproteinmL66 / 39S ribosomal protein S18-3 / mitochondrial / MRP-S18-3 / 39S ribosomal protein S18a / MRP-S18-a / ...39S ribosomal protein S18-3 / mitochondrial / MRP-S18-3 / 39S ribosomal protein S18a / MRP-S18-a / Mrps18a / S18mt-a / Large ribosomal subunit protein bS18a
Mass: 22224.078 Da / Num. of mol.: 1 / Source method: isolated from a natural source / Source: (natural) Homo sapiens (human) / References: UniProt: Q9NVS2
#85: Protein
LargeribosomalsubunitproteinmL65 / 39S ribosomal protein S30 / mitochondrial / MRP-S30 / S30mt / Large ribosomal subunit protein mS30 ...39S ribosomal protein S30 / mitochondrial / MRP-S30 / S30mt / Large ribosomal subunit protein mS30 / Programmed cell death protein 9
Mass: 50429.578 Da / Num. of mol.: 1 / Source method: isolated from a natural source / Source: (natural) Homo sapiens (human) / References: UniProt: Q9NP92
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