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RRG8 | Protein of unknown function; required for mitochondrial genome maintenance; null mutation results in a decrease in plasma membrane electron transport; Belongs to the RRG8 family. (277 aa) | ||||
GAL4 | Regulatory protein GAL4; DNA-binding transcription factor required for activating GAL genes; responds to galactose; repressed by Gal80p and activated by Gal3p. (881 aa) | ||||
COX1 | Subunit I of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; one of three mitochondrially-encoded subunits; Belongs to the heme-copper respiratory oxidase family. (534 aa) | ||||
ATP6 | Subunit a of the F0 sector of mitochondrial F1F0 ATP synthase; mitochondrially encoded; translation is specifically activated by Atp22p; ATP6 and ATP8 mRNAs are not translated in the absence of the F1 sector of ATPase; mutations in human ortholog MT-ATP6 are associated with neurodegenerative disorders such as Neurogenic Ataxia and Retinitis Pigmentosa (NARP), Leigh syndrome (LS), Charcot-Marie-Tooth (CMT), and ataxia telangiectasia. (259 aa) | ||||
COB | Cytochrome b; mitochondrially encoded subunit of the ubiquinol-cytochrome c reductase complex which includes Cobp, Rip1p, Cyt1p, Cor1p, Qcr2p, Qcr6p, Qcr7p, Qcr8p, Qcr9p, and Qcr10p. (385 aa) | ||||
COX2 | Subunit II of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; one of three mitochondrially-encoded subunits. (251 aa) | ||||
CYC3 | Cytochrome c heme lyase (holocytochrome c synthase); attaches heme to apo-cytochrome c (Cyc1p or Cyc7p) in mitochondrial intermembrane space; human homolog HCCS implicated in microphthalmia with linear skin defects (MLS), and can complement yeast null mutant. (269 aa) | ||||
FZO1 | 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; Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family. Mitofusin subfamily. (855 aa) | ||||
MRPL37 | Mitochondrial ribosomal protein of the large subunit. (105 aa) | ||||
SLM5 | Asparagine--tRNA ligase, mitochondrial; Mitochondrial asparaginyl-tRNA synthetase. (492 aa) | ||||
MSS2 | Protein MSS2, mitochondrial; Peripherally bound inner membrane protein of the mitochondrial matrix; involved in membrane insertion of C-terminus of Cox2p, interacts genetically and physically with Cox18p. (351 aa) | ||||
YDL129W | Uncharacterized protein YDL129W; Protein of unknown function; green fluorescent protein (GFP)-fusion protein localizes to the cytoplasm and the nucleus; YDL129W is not an essential gene; relative distribution to the nucleus increases upon DNA replication stress. (291 aa) | ||||
SRF1 | Regulator of phospholipase D (Spo14p); interacts with Spo14p and regulates its catalytic activity; capable of buffering the toxicity of C16:0 platelet activating factor, a lipid that accumulates intraneuronally in Alzheimer's patients. (437 aa) | ||||
RRG1 | Protein of unknown function; required for vacuolar acidification and mitochondrial genome maintenance; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies; Belongs to the RRG1 family. (365 aa) | ||||
PET100 | Protein PET100, mitochondrial; Chaperone that facilitates the assembly of cytochrome c oxidase; integral to the mitochondrial inner membrane; interacts with a subcomplex of subunits VII, VIIa, and VIII (Cox7p, Cox9p, and Cox8p) but not with the holoenzyme. (111 aa) | ||||
YDR114C | Uncharacterized protein YDR114C; Putative protein of unknown function; deletion mutant exhibits poor growth at elevated pH and calcium. (100 aa) | ||||
MRPL1 | Mitochondrial ribosomal protein of the large subunit. (285 aa) | ||||
RSM24 | Mitochondrial ribosomal protein of the small subunit. (319 aa) | ||||
CBS2 | Mitochondrial translational activator of the COB mRNA; interacts with translating ribosomes, acts on the COB mRNA 5'-untranslated leader. (389 aa) | ||||
MHR1 | Mitochondrial ribosomal protein of the large subunit; also involved in homologous recombination in mitochondria; required for recombination-dependent mtDNA partitioning; involved in stimulation of mitochondrial DNA replication in response to oxidative stress. (226 aa) | ||||
CYC7 | Cytochrome c isoform 2, expressed under hypoxic conditions; also known as iso-2-cytochrome c; electron carrier of the mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; protein abundance increases in response to DNA replication stress; CYC7 has a paralog, CYC1, that arose from the whole genome duplication. (113 aa) | ||||
YMR31 | 37S ribosomal protein YMR-31, mitochondrial; Subunit of the mitochondrial alpha-ketoglutarate dehydrogenase; recruits E3 subunit (Lpd1p) to the E1-E2 (Kgd1p, Kgd2p) core; has similarity to human mitochondrial ribosomal protein MRP-S36. (123 aa) | ||||
DOC1 | Anaphase-promoting complex subunit DOC1; Processivity factor; required for the ubiquitination activity of the anaphase promoting complex (APC), mediates the activity of the APC by contributing to substrate recognition; involved in cyclin proteolysis; contains a conserved DOC1 homology domain; Belongs to the APC10 family. (250 aa) | ||||
GTF1 | Glutamyl-tRNA(Gln) amidotransferase subunit F, mitochondrial; Subunit of the trimeric GatFAB AmidoTransferase(AdT) complex; involved in the formation of Q-tRNAQ; transposon insertion mutant is salt sensitive and null mutant has growth defects; non-tagged protein is detected in purified mitochondria. (183 aa) | ||||
CCM1 | Mitochondrial group I intron splicing factor CCM1; 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. (864 aa) | ||||
PET54 | Protein PET54; Mitochondrial inner membrane protein; binds to the 5' UTR of the COX3 mRNA to activate its translation together with Pet122p and Pet494p; also binds to the COX1 Group I intron AI5 beta to facilitate exon ligation during splicing. (293 aa) | ||||
MSH1 | DNA-binding protein of the mitochondria; involved in repair of mitochondrial DNA; has ATPase activity and binds to DNA mismatches; has homology to E. coli MutS; transcription is induced during meiosis. (959 aa) | ||||
MTG2 | GTPase MTG2, mitochondrial; Putative GTPase; member of the Obg family; peripheral protein of the mitochondrial inner membrane that associates with the large ribosomal subunit; required for mitochondrial translation, possibly via a role in ribosome assembly; Belongs to the TRAFAC class OBG-HflX-like GTPase superfamily. OBG GTPase family. (518 aa) | ||||
MRS1 | Splicing protein; required for splicing of two mitochondrial group I introns (BI3 in COB and AI5beta in COX1); forms a splicing complex, containing four subunits of Mrs1p and two subunits of the BI3-encoded maturase, that binds to the BI3 RNA; MRS1 has a paralog, CCE1, that arose from the whole genome duplication. (363 aa) | ||||
COX16 | Mitochondrial inner membrane protein; required for assembly of cytochrome c oxidase. (118 aa) | ||||
AIM22 | 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; Belongs to the LplA family. (409 aa) | ||||
COA3 | Mitochondrial protein required for cytochrome c oxidase assembly; also involved in translational regulation of Cox1p and prevention of Cox1p aggregation before assembly; located in the mitochondrial inner membrane. (85 aa) | ||||
CYC1 | Cytochrome c, isoform 1; also known as iso-1-cytochrome c; electron carrier of mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; CYC1 has a paralog, CYC7, that arose from the whole genome duplication; human homolog CYC1 can complement yeast null mutant; mutations in human CYC1 cause insulin-responsive hyperglycemia. (109 aa) | ||||
MGM101 | Mitochondrial genome maintenance protein MGM101; Protein with a role in mitochondrial DNA recombinational repair; also involved in interstrand cross-link repair; binds to and catalyzes the annealing of single-stranded mtDNA; oligomerizes to form rings and filaments; related to Rad52-type recombination proteins, with limited overall similarity but sharing conserved functionally important residues; component of the mitochondrial nucleoid, required for the repair of oxidative mtDNA damage and mitochondrial genome maintenance; Belongs to the MGM101 family. (269 aa) | ||||
MRP17 | Mitochondrial ribosomal protein of the small subunit; MRP17 exhibits genetic interactions with PET122, encoding a COX3-specific translational activator. (131 aa) | ||||
APN1 | DNA-(apurinic or apyrimidinic site) lyase 1; Major apurinic/apyrimidinic endonuclease; 3'-repair diesterase; involved in repair of DNA damage by oxidation and alkylating agents; also functions as a 3'-5' exonuclease to repair 7,8-dihydro-8-oxodeoxyguanosine; genetically interacts with NTG1 to maintain mitochondrial genome integrity; Belongs to the AP endonuclease 2 family. (367 aa) | ||||
MRPL38 | Mitochondrial ribosomal protein of the large subunit; appears as two protein spots (YmL34 and YmL38) on two-dimensional SDS gels; protein abundance increases in response to DNA replication stress. (138 aa) | ||||
CBT1 | Cytochrome b termination protein 1; Protein involved in 5' RNA end processing; substrates include mitochondrial COB, 15S_rRNA, and RPM1 transcripts; may also have a role in 3' end processing of the COB pre-mRNA; displays genetic interaction with cell cycle-regulated kinase Dbf2p. (271 aa) | ||||
COX19 | Protein required for cytochrome c oxidase assembly; located in the cytosol and mitochondrial intermembrane space; putative copper metallochaperone that delivers copper to cytochrome c oxidase; contains twin cysteine-x9-cysteine motifs. (98 aa) | ||||
IRC19 | Increased recombination centers protein 19; Protein of unknown function; YLL033W is not an essential gene but mutant is defective in spore formation; null mutant displays increased levels of spontaneous Rad52p foci. (230 aa) | ||||
GEP5 | Genetic interactor of prohibitin 5, mitochondrial; Protein of unknown function; required for mitochondrial genome maintenance; detected in highly purified mitochondria in high-throughput studies; null mutant has decreased levels of cardiolipin and phosphatidylethanolamine; Belongs to the GEP5 family. (293 aa) | ||||
MSS51 | Protein MSS51, mitochondrial; Specific translational activator for the mitochondrial COX1 mRNA; loosely associated with the matrix face of the mitochondrial inner membrane; localizes to vacuole membrane in response to H2O2; influences both COX1 mRNA translation and Cox1p assembly into cytochrome c oxidase; binds to heme B, which may be a mechanism for sensing oxygen levels in order to regulate cytochrome c oxidase biogenesis. (436 aa) | ||||
ACO1 | Aconitate hydratase, mitochondrial; Aconitase; required for the tricarboxylic acid (TCA) cycle and also independently required for mitochondrial genome maintenance; component of the mitochondrial nucleoid; mutation leads to glutamate auxotrophy; human homolog ACO2 can complement yeast null mutant. (778 aa) | ||||
HMG2 | HMG-CoA reductase; converts HMG-CoA to mevalonate, a rate-limiting step in sterol biosynthesis; one of two isozymes; overproduction induces assembly of peripheral ER membrane arrays and short nuclear-associated membrane stacks; forms foci at nuclear periphery upon DNA replication stress; HMG2 has a paralog, HMG1, that arose from the whole genome duplication; human homolog HMGCR can complement yeast hmg2 mutant. (1045 aa) | ||||
RAD52 | DNA repair and recombination protein RAD52; Protein that stimulates strand exchange; stimulates strand exchange by facilitating Rad51p binding to single-stranded DNA; anneals complementary single-stranded DNA; involved in the repair of double-strand breaks in DNA during vegetative growth and meiosis and UV induced sister chromatid recombination; Belongs to the RAD52 family. (471 aa) | ||||
PIF1 | DNA helicase, potent G-quadruplex DNA binder/unwinder; possesses strand annealing activity; promotes DNA synthesis during break-induced replication; important for crossover recombination; translation from different start sites produces mitochondrial and nuclear forms; nuclear form is a catalytic inhibitor of telomerase; mitochondrial form involved in DNA repair and recombination; mutations affect Zn, Fe homeostasis; regulated by Rad53p-dependent phosphorylation in rho0 cells. (859 aa) | ||||
MTF1 | Mitochondrial RNA polymerase specificity factor; has structural similarity to S-adenosylmethionine-dependent methyltransferases and functional similarity to bacterial sigma-factors; Mtf1p interacts with and stabilizes the Rpo41p-promoter complex, enhancing DNA bending and melting to facilitate pre-initiation open complex formation; Belongs to the class I-like SAM-binding methyltransferase superfamily. rRNA adenine N(6)-methyltransferase family. (341 aa) | ||||
AEP2 | ATPase expression protein 2, mitochondrial; Mitochondrial protein; likely involved in translation of the mitochondrial OLI1 mRNA; exhibits genetic interaction with the OLI1 mRNA 5'-untranslated leader; Belongs to the AEP2 family. (580 aa) | ||||
HER2 | Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial; Subunit of the trimeric GatFAB AmidoTransferase(AdT) complex; involved in the formation of Q-tRNAQ; required for remodeling of ER caused by Hmg2p overexpression; similar to bacterial GatA glutamyl-tRNA amidotransferase. (464 aa) | ||||
YNL184C | Uncharacterized protein YNL184C; Protein of unknown function; expressed at both mRNA and protein levels. (108 aa) | ||||
KAR1 | Cell division control protein KAR1; Protein involved in karyogamy and spindle pole body duplication; involved in karyogamy during mating; involved in spindle pole body duplication during mitosis; localizes to the half-bridge of the spindle pole body; interacts with Spc72p during karyogamy; also interacts with Cdc31p; essential gene. (433 aa) | ||||
RRG9 | Required for respiratory growth protein 9, mitochondrial; Protein of unknown function; null mutant lacks mitochondrial DNA and cannot grow on glycerol; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies. (214 aa) | ||||
PET494 | Mitochondrial translational activator specific for the COX3 mRNA; acts together with Pet54p and Pet122p; located in the mitochondrial inner membrane. (489 aa) | ||||
SDH5 | Succinate dehydrogenase assembly factor 2, mitochondrial; Protein required for flavinylation of Sdh1p; binds to Sdh1p and promotes FAD cofactor attachment, which is necessary for succinate dehydrogenase (SDH) complex assembly and activity; mutations in human ortholog PGL2 are associated with neuroendocrine tumors (paraganglioma). (162 aa) | ||||
ATG34 | Autophagy-related protein 34; Receptor protein involved in selective autophagy during starvation; specifically involved in the transport of cargo protein alpha-mannosidase (Ams1p); Atg19p paralog. (412 aa) | ||||
HMI1 | Mitochondrial inner membrane localized ATP-dependent DNA helicase; required for the maintenance of the mitochondrial genome; not required for mitochondrial transcription; has homology to E. coli helicase uvrD. (706 aa) | ||||
COQ3 | O-methyltransferase; catalyzes two different O-methylation steps in ubiquinone (Coenzyme Q) biosynthesis; component of a mitochondrial ubiquinone-synthesizing complex; phosphoprotein; Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family. (312 aa) | ||||
MGM1 | 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. (881 aa) | ||||
MRPS16 | Mitochondrial ribosomal protein of the small subunit. (121 aa) | ||||
RRG7 | Required for respiratory growth protein 7, mitochondrial; Protein of unknown function; green fluorescent protein (GFP)-fusion protein localizes to the mitochondrion; deletion confers sensitivity to 4-(N-(S-glutathionylacetyl)amino) phenylarsenoxide (GSAO); YOR305W is not an essential gene. (242 aa) | ||||
COX10 | Protoheme IX farnesyltransferase, mitochondrial; Heme A:farnesyltransferase; catalyzes first step in conversion of protoheme to heme A prosthetic group required for cytochrome c oxidase activity; human ortholog COX10 can complement yeast cox10 null mutant; human ortholog COX10 is associated with mitochondrial disorders; Belongs to the UbiA prenyltransferase family. (462 aa) | ||||
ATP4 | Subunit b of the stator stalk of mitochondrial F1F0 ATP synthase; ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; contributes to the oligomerization of the complex, which in turn determines the shape of inner membrane cristae; phosphorylated; Belongs to the eukaryotic ATPase B chain family. (244 aa) |