Sequence Description Alias PCC hrr YBL022C ATP-dependent Lon protease; involved in degradation of misfolded proteins in mitochondria; required for biogenesis and maintenance of mitochondria [Source:SGD;Acc:S000000118] 0.8139135230202907 1 YPR024W Catalytic subunit of i-AAA protease complex; complex is located in mitochondrial inner membrane; responsible for degradation of unfolded or misfolded mitochondrial gene products; serves as nonconventional translocation motor to pull PNPase into intermembrane space; also has role in intermembrane space protein folding; mutation causes elevated rate of mitochondrial turnover; human homolog YME1L1 can complement yeast null mutant [Source:SGD;Acc:S000006228] 0.7420774955720686 5 YOR211C Mitochondrial GTPase, present in complex with Ugo1p and Fzo1p; required for mitochondrial morphology, fusion, and genome maintenance; promotes membrane bending; exists as long and short form with different distributions; ratio of long to short forms is regulated by Psd1p; homolog of human OPA1 involved in autosomal dominant optic atrophy [Source:SGD;Acc:S000005737] 0.7328918753062118 64 YMR098C Mitochondrial protein required for the stability of Oli1p (Atp9p) mRNA; also required for the Oli1p ring formation; YMR098C is not an essential gene [Source:SGD;Acc:S000004704] 0.7238079863553325 58 YBR179C Mitofusin; integral membrane protein involved in mitochondrial outer membrane tethering and fusion; role in mitochondrial genome maintenance; efficient tethering and degradation of Fzo1p requires an intact N-terminal GTPase domain; targeted for destruction by the ubiquitin ligase SCF-Mdm30p and the cytosolic ubiquitin-proteasome system [Source:SGD;Acc:S000000383] 0.7206657325146799 30 YER080W Protein of unknown function; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies; null mutant displays elevated frequency of mitochondrial genome loss [Source:SGD;Acc:S000000882] 0.7205210492475307 31 YIL005W ER protein with chaperone and co-chaperone activity; involved in retention of resident ER proteins; has a role in recognizing proteins targeted for ER-associated degradation (ERAD), member of the protein disulfide isomerase family [Source:SGD;Acc:S000001267] 0.715804312696202 86 YLR382C Mitochondrial leucyl-tRNA synthetase; also has direct role in splicing of several mitochondrial group I introns; indirectly required for mitochondrial genome maintenance; human homolog LARS2 can complement yeast null mutant, and is implicated in Perrault syndrome [Source:SGD;Acc:S000004374] 0.7089992584761313 21 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] 0.7016142519060161 71 YHR027C Non-ATPase base subunit of the 19S RP of the 26S proteasome; may participate in the recognition of several ligands of the proteasome; contains a leucine-rich repeat (LRR) domain, a site for protein-protein interactions; RP is the acronym for regulatory particle [Source:SGD;Acc:S000001069] 0.6987668226375646 18 YDR430C Lysine-specific metalloprotease of the pitrilysin family; metalloprotease of the intermembrane space; degrades proteins and presequence peptides cleaved from imported proteins; required for normal mitochondrial morphology [Source:SGD;Acc:S000002838] 0.6963142688717302 50 YJL112W Peripheral protein of cytosolic face of mitochondrial outer membrane; required for mitochondrial fission; interacts with Fis1p and with the self-assembled oligomeric form of the dynamin-related GTPase Dnm1p; contains WD repeats; MDV1 has a paralog, CAF4, that arose from the whole genome duplication [Source:SGD;Acc:S000003648] 0.6934277237854679 46 YOR354C Multicopy suppressor of HER2 involved in mitochondrial translation; mutant is defective in directing meiotic recombination events to homologous chromatids [Source:SGD;Acc:S000005881] 0.6928303342508612 60 YGR186W TFIIF (Transcription Factor II) largest subunit; involved in both transcription initiation and elongation of RNA polymerase II; homologous to human RAP74 [Source:SGD;Acc:S000003418] 0.6926912878245919 33 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] 0.6924925032647735 94 YMR302C Integral inner mitochondrial membrane protein; role in maintaining mitochondrial nucleoid structure and number; mutants exhibit an increased rate of mitochondrial DNA escape; shows some sequence similarity to exonucleases [Source:SGD;Acc:S000004917] 0.6897071420350604 55 YNL121C Component of the TOM (translocase of outer membrane) complex; involved in the recognition and initial import steps for all mitochondrially directed proteins; acts as a receptor for incoming precursor proteins; TOM70 has a paralog, TOM71, that arose from the whole genome duplication [Source:SGD;Acc:S000005065] 0.6880765093611667 35 YDR515W RNA binding protein that associates with polysomes; may be involved in regulating mRNA translation; involved in the copper-dependent mineralization of copper sulfide complexes on cell surface in cells cultured in copper salts; SLF1 has a paralog, SRO9, that arose from the whole genome duplication; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000002923] 0.6878473752414691 46 YPL104W Mitochondrial aspartyl-tRNA synthetase; required for acylation of aspartyl-tRNA; yeast and bacterial aspartyl-, asparaginyl-, and lysyl-tRNA synthetases contain regions with high sequence similarity, suggesting a common ancestral gene [Source:SGD;Acc:S000006025] 0.6863370051838703 66 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] 0.6840600138507399 69 YLR289W Mitochondrial matrix GTPase; associates with mitochondrial ribosomes; important for translation under temperature and nutrient stress; may have a role in translational fidelity; similar to bacterial LepA elongation factor [Source:SGD;Acc:S000004280] 0.6836367512637962 81 YKL195W Import and assembly protein in mitochondrial intermembrane space; component of MIA pathway which mediates import and oxidative folding of substrates including small proteins containing twin cysteine motifs; acts in concert with Erv1p, which oxidizes the cysteine residues of Mia40p to comprise a disulfide relay system that catalyzes import; also mediates folding of Atp23p via a chaperone-like activity; forms a dimer that binds iron-sulfur cluster in vitro [Source:SGD;Acc:S000001678] 0.6830964210559474 81 YKL054C RNAPII degradation factor; forms a complex with Rad26p in chromatin, enables ubiquitination and proteolysis of RNAPII present in an elongation complex; mutant is deficient in Zip1p loading onto chromosomes during meiosis [Source:SGD;Acc:S000001537] 0.681738643505026 100 YOL023W Mitochondrial translation initiation factor 2 [Source:SGD;Acc:S000005383] 0.6805686811010377 65 YJL054W Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane [Source:SGD;Acc:S000003590] 0.679110999915947 46 YLL001W Dynamin-related GTPase involved in mitochondrial organization; required for mitochondrial fission and inheritance; self assembles on the cytoplasmic face of mitochondrial tubules at sites where division will occur; participates in endocytosis and regulates peroxisome fission along with Vps1p; mutants in the human ortholog DNM1L, which mediates mitochondrial fission, peroxisomal division, autophagy, and mitophagy, are associated with slowly progressive infantile encephalopathy [Source:SGD;Acc:S000003924] 0.6787207836263712 47 YMR115W Subunit of the mitochondrial (mt) i-AAA protease supercomplex; i-AAA degrades misfolded mitochondrial proteins; forms a subcomplex with Mgr1p that binds to substrates to facilitate proteolysis; required for growth of cells lacking mtDNA [Source:SGD;Acc:S000004721] 0.6712854910965784 86 YPL040C Mitochondrial isoleucyl-tRNA synthetase; null mutant is deficient in respiratory growth; human homolog IARS2 implicated in mitochondrial diseases, can partially complement yeast null mutant [Source:SGD;Acc:S000005961] 0.6697965002753723 66 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] 0.6653564922893834 74 YIL075C Subunit of the 26S proteasome; substrate of the N-acetyltransferase Nat1p; protein abundance increases in response to DNA replication stress [Source:SGD;Acc:S000001337] 0.6606563374788523 85 YLR069C Mitochondrial elongation factor involved in translational elongation [Source:SGD;Acc:S000004059] 0.6569357534852136 93 YOL027C Mitochondrial protein; forms a complex with Mba1p to facilitate recruitment of mRNA-specific translational activators to ribosomes; roles in protein export and K+/H+ exchange; human ortholog Letm1 implicated in Wolf-Hirschhorn syndrome [Source:SGD;Acc:S000005387] 0.655030427747809 95