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| GON7 | EKC/KEOPS complex subunit GON7; Component of the EKC/KEOPS complex that is required for the formation of a threonylcarbamoyl group on adenosine at position 37 (t(6)A37) in tRNAs that read codons beginning with adenine. The complex is probably involved in the transfer of the threonylcarbamoyl moiety of threonylcarbamoyl-AMP (TC-AMP) to the N6 group of A37. GON7 likely plays a supporting role to the catalytic subunit OSGEP in the complex. (100 aa) | ||||
| TPRKB | TP53RK binding protein; Belongs to the CGI121/TPRKB family. (175 aa) | ||||
| OSGEPL1 | Probable tRNA N6-adenosine threonylcarbamoyltransferase, mitochondrial; Required for the formation of a threonylcarbamoyl group on adenosine at position 37 (t(6)A37) in mitochondrial tRNAs that read codons beginning with adenine. Probably involved in the transfer of the threonylcarbamoyl moiety of threonylcarbamoyl-AMP (TC-AMP) to the N6 group of A37. Involved in mitochondrial genome maintenance. (414 aa) | ||||
| TRIT1 | tRNA dimethylallyltransferase; Catalyzes the transfer of a dimethylallyl group onto the adenine at position 37; Belongs to the IPP transferase family. (467 aa) | ||||
| HSD17B10 | 3-hydroxyacyl-CoA dehydrogenase type-2; Mitochondrial dehydrogenase that catalyzes the beta-oxidation at position 17 of androgens and estrogens and has 3-alpha- hydroxysteroid dehydrogenase activity with androsterone. Catalyzes the third step in the beta-oxidation of fatty acids. Carries out oxidative conversions of 7-alpha-OH and 7-beta-OH bile acids. Also exhibits 20- beta-OH and 21-OH dehydrogenase activities with C21 steroids. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD). Essential for structura [...] (261 aa) | ||||
| MTO1 | Mitochondrial translation optimization 1 homolog (S. cerevisiae). (692 aa) | ||||
| CDK5RAP1 | CDK5 regulatory subunit associated protein 1. (597 aa) | ||||
| TRMT61B | tRNA methyltransferase 61B. (500 aa) | ||||
| LOC101903778 | EKC/KEOPS complex subunit LAGE3-like. (147 aa) | ||||
| LAGE3 | L antigen family member 3. (155 aa) | ||||
| LOC112445102 | Uncharacterized protein. (147 aa) | ||||
| TRMT5 | tRNA (guanine(37)-N1)-methyltransferase; Involved in mitochondrial tRNA methylation (By similarity). Specifically methylates the N1 position of guanosine-37 in various tRNAs. Methylation is not dependent on the nature of the nucleoside 5' of the target nucleoside. This is the first step in the biosynthesis of wybutosine (yW), a modified base adjacent to the anticodon of tRNAs and required for accurate decoding; Belongs to the class I-like SAM-binding methyltransferase superfamily. TRM5/TYW2 family. (507 aa) | ||||
| LOC112445126 | Uncharacterized protein. (170 aa) | ||||
| OSGEP | Probable tRNA N6-adenosine threonylcarbamoyltransferase; Component of the EKC/KEOPS complex that is required for the formation of a threonylcarbamoyl group on adenosine at position 37 (t(6)A37) in tRNAs that read codons beginning with adenine. The complex is probably involved in the transfer of the threonylcarbamoyl moiety of threonylcarbamoyl-AMP (TC-AMP) to the N6 group of A37. OSGEP likely plays a direct catalytic role in this reaction, but requires other protein(s) of the complex to fulfill this activity. (335 aa) | ||||
| ENSBTAP00000061226 | Uncharacterized protein. (136 aa) | ||||
| YRDC | YrdC domain-containing protein, mitochondrial; May regulate the activity of some transporters. (327 aa) | ||||
| TP53RK | TP53 regulating kinase. (253 aa) | ||||
| TRMT10C | tRNA methyltransferase 10 homolog C; Mitochondrial tRNA N(1)-methyltransferase involved in mitochondrial tRNA maturation. Component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and PRORP/MRPP3, which cleaves tRNA molecules in their 5'-ends. Together with HSD17B10/MRPP2, forms a subcomplex of the mitochondrial ribonuclease P, named MRPP1-MRPP2 subcomplex, which displays functions that are independent of the ribonuclease P activity. The MRPP1-MRPP2 subcomplex catalyzes the formation of N(1)-methylguanine and N(1)-methyladenine at position 9 (m1G9 a [...] (420 aa) | ||||
| RPUSD4 | Mitochondrial RNA pseudouridine synthase RPUSD4; Catalyzes uridine to pseudouridine isomerization (pseudouridylation) of different mitochondrial RNA substrates. Acts on position 1397 in 16S mitochondrial ribosomal RNA (16S mt-rRNA). This modification is required for the assembly of 16S mt-rRNA into a functional mitochondrial ribosome. Acts on position 39 in mitochondrial tRNA(Phe). As a component of a functional protein-RNA module, consisting of RCC1L, NGRN, RPUSD3, RPUSD4, TRUB2, FASTKD2 and 16S mt- rRNA, controls 16S mt-rRNA abundance and is required for intra- mitochondrial translation. (377 aa) | ||||