Sequence Description Alias PCC hrr YPL029W ATP-dependent RNA helicase; component of the mitochondrial degradosome along with the RNase Dss1p; the degradosome associates with the ribosome and mediates RNA turnover; also required during splicing of the COX1 AI5_beta intron; expression of a processed form of human homolog SUPV3L1 carrying an N-terminal deletion of 46 amino acids rescues yeast suv3 null mutant [Source:SGD;Acc:S000005950] 0.7540970389054146 14 YGR150C Mitochondrial 15S rRNA-binding protein; required for intron removal of COB and COX1 pre-mRNAs; has separable roles in stabilizing mitochondrial 15S rRNA and in maturation of the COB and COX1 mRNAs; contains pentatricopeptide repeat (PPR) motifs; mutant is respiratory deficient and has defective plasma membrane electron transport [Source:SGD;Acc:S000003382] 0.7458738582307642 26 YOR205C Protein required for mitochondrial ribosome small subunit biogenesis; null mutant is defective in respiration and in maturation of 15S rRNA; protein is localized to the mitochondrial inner membrane; null mutant interacts synthetically with prohibitin (Phb1p) [Source:SGD;Acc:S000005731] 0.7243595479105617 65 YGL064C Mitochondrial ATP-dependent RNA helicase of the DEAD-box family; required for assembly of the large subunit of mitochondrial ribosomes; binds to the large subunit rRNA, 21S_rRNA; localizes to the matrix face of the mitochondrial inner membrane and associates with the large subunit precursor and with mature ribosomes [Source:SGD;Acc:S000003032] 0.7207491885804537 10 YGL107C Mitochondrial protein required for respiratory growth; mutant phenotype and genetic interactions suggest a role in delivering mt mRNAs to ribosomes; located on matrix face of the inner membrane and loosely associated with mitoribosomes; RMD9 has a paralog, YBR238C, that arose from the whole genome duplication [Source:SGD;Acc:S000003075] 0.7190355695479137 49 YPL072W Deubiquitinating enzyme anchored to the outer mitochondrial membrane; probably not important for general mitochondrial functioning, but may perform a more specialized function at mitochondria [Source:SGD;Acc:S000005993] 0.7047279093222412 58 YKL155C Mitochondrial ribosomal protein of the small subunit; also predicted to be an S-adenosylmethionine-dependent RNA methyltransferase [Source:SGD;Acc:S000001638] 0.7039119759950165 55 YMR282C Mitochondrial protein; likely involved in translation of the mitochondrial OLI1 mRNA; exhibits genetic interaction with the OLI1 mRNA 5'-untranslated leader [Source:SGD;Acc:S000004895] 0.6989466684158275 66 YPR034W Component of both the SWI/SNF and RSC chromatin remodeling complexes; actin-related protein involved in transcriptional regulation [Source:SGD;Acc:S000006238] 0.6944358559340646 74 YDR514C Protein of unknown function that localizes to mitochondria; overexpression affects endocytic protein trafficking; YDR514C has a paralog, GFD2, that arose from the whole genome duplication [Source:SGD;Acc:S000002922] 0.6903100635840244 20 YDR194C Mitochondrial transcription elongation factor; DEAD-box protein; required for efficient splicing of mitochondrial Group I and II introns; non-polar RNA helicase that also facilities strand annealing; promotes RNA folding by stabilizing an early assembly intermediate [Source:SGD;Acc:S000002602] 0.6858406497472253 18 YGR084C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000003316] 0.6819421623198767 66 YHR091C Mitochondrial arginyl-tRNA synthetase; mutations in human ortholog are associated with pontocerebellar hypoplasia type 6; MSR1 has a paralog, YDR341C, that arose from the whole genome duplication [Source:SGD;Acc:S000001133] 0.6784509350233022 85 YJL054W Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane [Source:SGD;Acc:S000003590] 0.6749382527556873 34 YHL004W Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000000996] 0.6734652264641376 71 YPR125W Mitochondrial inner membrane protein; exposed to the mitochondrial matrix; associates with mitochondrial ribosomes; NOT required for respiratory growth; homolog of human Letm1, a protein implicated in Wolf-Hirschhorn syndrome [Source:SGD;Acc:S000006329] 0.6718368247803322 25 YIL021W RNA polymerase II third largest subunit B44; part of central core; similar to prokaryotic alpha subunit [Source:SGD;Acc:S000001283] 0.6707675632113191 47 YJL180C Assembly factor for F1 sector of mitochondrial F1F0 ATP synthase; conserved protein; required for assembly of alpha and beta subunits into F1 sector of mitochondrial F1F0 ATP synthase; human homolog ATPAF2 can complement yeast atp12 mutant; mutation of human homolog reduces active ATP synthase levels and is associated with the disorder ATPAF2 deficiency [Source:SGD;Acc:S000003716] 0.667572171479426 78 YOL023W Mitochondrial translation initiation factor 2 [Source:SGD;Acc:S000005383] 0.6630618104800818 82 YOR334W Mitochondrial inner membrane Mg(2+) channel; required for maintenance of intramitochondrial Mg(2+) concentrations at the correct level to support splicing of group II introns; similar to bacterial CorA [Source:SGD;Acc:S000005861] 0.6531223207972225 61 YGL172W FG-nucleoporin component of central core of the nuclear pore complex; contributes directly to nucleocytoplasmic transport and maintenance of the nuclear pore complex (NPC) permeability barrier; found in stable complex with Nic96p and two other FG-nucleoproteins (Nsp1p and Nup57p) [Source:SGD;Acc:S000003140] 0.6499014272441127 99 YPL050C Subunit of Golgi mannosyltransferase complex; this complex mediates elongation of the polysaccharide mannan backbone; forms a separate complex with Van1p that is also involved in backbone elongation; this complex also contains Anp1p, Mnn10p, Mnn11p, and Hoc1p [Source:SGD;Acc:S000005971] 0.6454602857114534 91 YDL202W Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2 [Source:SGD;Acc:S000002361] 0.6437530087124528 79 YMR024W Mitochondrial ribosomal protein of the large subunit; located in close proximity to the polypeptide exit channel of the ribosome; mutations in human homolog MRPL44 cause childhood cardiomyopathy; human MRPL44 deficiency results in inefficient assembly of the mitochondrial ribosome, and in tissue-specific respiratory chain deficiency, manifesting as either Complex I+Complex IV or Complex IV deficiency, depending on a cell type [Source:SGD;Acc:S000004626] 0.6413875978159861 91 YHL014C Protein of unknown function; has weak similarity to E. coli GTP-binding protein gtp1; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000001006] 0.6405371574558572 48 YIL093C Mitochondrial ribosomal protein of the small subunit [Source:SGD;Acc:S000001355] 0.6403592946832876 72 YML025C Mitochondrial ribosomal protein of the large subunit; has similarity to E. coli L4 ribosomal protein and human mitoribosomal MRP-L4 protein; essential for viability, unlike most other mitoribosomal proteins [Source:SGD;Acc:S000004487] 0.6403027888541268 99 YGR169C tRNA:pseudouridine synthase; catalyzes the conversion of uridine to pseudouridine at position 31 in cytoplasmic and mitochondrial tRNAs; mutation of Asp168 to Ala abolishes enzyme activity; not essential for viability [Source:SGD;Acc:S000003401] 0.6385177744524058 55 YMR166C Transporter of the mitochondrial inner membrane that exports magnesium; involved in mitochondrial Mg2+ homeostasis; has similarity to human mitochondrial ATP-Mg/Pi carriers [Source:SGD;Acc:S000004776] 0.6287264280613662 61 YNR020C Putative metalloprotease of the mitochondrial inner membrane; required for processing of Atp6p; has an additional role in assembly of the F0 sector of the F1F0 ATP synthase complex; substrate of the Mia40p-Erv1p disulfide relay system, and folding is assisted by Mia40p [Source:SGD;Acc:S000005303] 0.619667766225613 79 YOL033W Mitochondrial glutamyl-tRNA synthetase; predicted to be palmitoylated [Source:SGD;Acc:S000005393] 0.6192221553391668 81