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] 0.7440619970199593 23 YPL063W Essential component of the TIM23 complex; acts as receptor for the translocase of the inner mitochondrial membrane (TIM23) complex guiding incoming precursors from the TOM complex; may control the gating of the Tim23p-Tim17p channel [Source:SGD;Acc:S000005984] 0.740235069697856 2 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] 0.7390097620480616 33 YJL081C Nuclear actin-related protein involved in chromatin remodeling; component of chromatin-remodeling enzyme complexes [Source:SGD;Acc:S000003617] 0.7298419963521784 9 YKL155C Mitochondrial ribosomal protein of the small subunit; also predicted to be an S-adenosylmethionine-dependent RNA methyltransferase [Source:SGD;Acc:S000001638] 0.7259404470877688 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] 0.7212898077103522 72 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] 0.7186617770305669 28 YNL137C Mitochondrial ribosomal component of the small subunit [Source:SGD;Acc:S000005081] 0.7143194924175476 78 YGR178C Component of glucose deprivation induced stress granules; involved in P-body-dependent granule assembly; similar to human ataxin-2; interacts with Pab1p to regulate mRNA polyadenylation; interacts with Mkt1p to regulate HO translation; protein increases in abundance and relative distribution to the nucleus increases upon DNA replication stress [Source:SGD;Acc:S000003410] 0.7074610051074143 74 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] 0.7065549179744995 22 YIL022W Essential component of the TIM23 complex; tethers the import motor and regulatory factors (PAM complex) to the translocation channel (Tim23p-Tim17p core complex); TIM23 complex is short for the translocase of the inner mitochondrial membrane [Source:SGD;Acc:S000001284] 0.7029945410597472 20 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] 0.7023109452029888 76 YJL014W Subunit of the cytosolic chaperonin Cct ring complex; related to Tcp1p, required for the assembly of actin and tubulins in vivo; capable of binding Q/N rich proteins and mediating their folding [Source:SGD;Acc:S000003551] 0.700945563095822 21 YPL101W Subunit of hexameric RecA-like ATPase Elp456 Elongator subcomplex; which is required for modification of wobble nucleosides in tRNA; required for Elongator structural integrity; null mutation is functionally complemented by human ELP4 [Source:SGD;Acc:S000006022] 0.7003398934809852 24 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] 0.6962967329534167 19 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] 0.6953219818294207 86 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] 0.6950512221041424 21 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] 0.6931780563981828 29 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] 0.6925695137441448 24 YLR253W Mitochondrial protein of unknown function involved in lipid homeostasis; associates with mitochondrial ribosome; integral membrane protein that localizes to the mitochondrial inner membrane; involved in mitochondrial morphology; non-essential gene which interacts genetically with MDM10, and other members of the ERMES complex; transcription is periodic during the metabolic cycle; homologous to human aarF domain containing kinase, ADCK1 [Source:SGD;Acc:S000004243] 0.6901042361330169 79 YLR163C Beta subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins [Source:SGD;Acc:S000004153] 0.6889826708691117 60 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] 0.6866951763566563 68 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] 0.6857131187819111 88 YMR089C Mitochondrial inner membrane m-AAA protease component; mediates degradation of misfolded or unassembled proteins; also required for correct assembly of mitochondrial enzyme complexes; overexpression of human AFG3L2 complements respiratory defect of yeast afg3 yta12 double null mutation, but overexpression of disease-associated AFG3L2 variants does not; expression of both human SPG7 (paraplegin) and AFG3L2 complements yeast yta12 afg3 double mutation [Source:SGD;Acc:S000004695] 0.679110999915947 46 YBR192W Mitochondrial pyrimidine nucleotide transporter; imports pyrimidine nucleoside triphosphates and exports pyrimidine nucleoside monophosphates; member of the mitochondrial carrier family [Source:SGD;Acc:S000000396] 0.6749382527556873 34 YGR171C Mitochondrial methionyl-tRNA synthetase (MetRS); functions as a monomer in mitochondrial protein synthesis; functions similarly to cytoplasmic MetRS although the cytoplasmic form contains a zinc-binding domain not found in Msm1p [Source:SGD;Acc:S000003403] 0.6745930833262254 70 YGR007W Ethanolamine-phosphate cytidylyltransferase; catalyzes the second step of phosphatidylethanolamine biosynthesis; involved in the maintenance of plasma membrane; similar to mammalian CTP: phosphocholine cytidylyl-transferases; inability of the null mutant to synthesize phosphatidylethanolamine and phosphatidylcholine from ethanolamine is functionally complemented by human PCYT2 [Source:SGD;Acc:S000003239] 0.6745743369143632 36 YMR005W TFIID subunit (48 kDa); involved in RNA polymerase II transcription initiation; potential Cdc28p substrate [Source:SGD;Acc:S000004607] 0.6741374604885434 99 YJR076C Component of the septin ring that is required for cytokinesis; present at the ends of rod-like septin hetero-oligomers; C-terminal extension is important for recruitment of Bni5p to the mother-bud neck, which in turn is required for Myo1p recruitment and cytokinesis; septin rings at the mother-bud neck act as scaffolds for recruiting cell division factors and as barriers to prevent diffusion of specific proteins between mother and daughter cells [Source:SGD;Acc:S000003837] 0.6737373162121695 77 YLR090W Chaperone with a role in facilitating mitochondrial protein import; ascomycete-specific member of the DnaJ-like family, closely related to Ydj1p; predicted to be C-terminally prenylated; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies [Source:SGD;Acc:S000004080] 0.6736412714627021 39 YDR292C Signal recognition particle (SRP) receptor alpha subunit; contain GTPase domains; involved in SRP-dependent protein targeting; interacts with the beta subunit, Srp102p [Source:SGD;Acc:S000002700] 0.6705540799399643 51 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] 0.670396283144999 47 YLR427W Cytoplasmic protein of unknown function; induced in response to mycotoxin patulin; ubiquitinated protein similar to the human ring finger motif protein RNF10; predicted to be involved in repair of alkylated DNA due to interaction with MAG1 [Source:SGD;Acc:S000004419] 0.6693495834594325 85 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] 0.6692624568754857 74 YHR024C Alpha subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins [Source:SGD;Acc:S000001066] 0.6687037384446728 52 YNR029C Putative protein of unknown function; deletion confers reduced fitness in saline [Source:SGD;Acc:S000005312] 0.66796373752771 55 YNL005C Mitochondrial ribosomal protein of the large subunit [Source:SGD;Acc:S000004950] 0.6627189844677396 97 YDR061W Protein with similarity to ABC transporter family members; lacks predicted membrane-spanning regions; transcriptionally activated by Yrm1p along with genes involved in multidrug resistance [Source:SGD;Acc:S000002468] 0.6607211317845773 69 YJL046W Putative lipoate-protein ligase; required along with Lip2 and Lip5 for lipoylation of Lat1p and Kgd2p; similar to E. coli LplA; null mutant displays reduced frequency of mitochondrial genome loss [Source:SGD;Acc:S000003582] 0.6595176930178137 82 YML115C Component of the mannan polymerase I; complex contains Van1p and Mnn9p and is involved in the first steps of mannan synthesis; mutants are vanadate-resistant [Source:SGD;Acc:S000004583] 0.6594572404547445 71 YOR061W Alpha' catalytic subunit of casein kinase 2 (CK2); CK2 is a Ser/Thr protein kinase with roles in cell growth and proliferation; CK2, comprised of CKA1, CKA2, CKB1 and CKB2, has many substrates including transcription factors and all RNA polymerases; protein abundance increases in response to DNA replication stress; regulates Fkh1p-mediated donor preference during mating-type switching [Source:SGD;Acc:S000005587] 0.6580181868021973 73 YOL023W Mitochondrial translation initiation factor 2 [Source:SGD;Acc:S000005383] 0.6556899658711285 95 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] 0.6513249455981551 96 YGL129C Mitochondrial ribosomal protein of the small subunit; has similarity to mammalian apoptosis mediator proteins; null mutation prevents induction of apoptosis by overproduction of metacaspase Mca1p [Source:SGD;Acc:S000003097] 0.6470953977997305 100