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] 0.7748594765917889 9 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] 0.7576237882524752 7 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] 0.7453228792061075 36 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] 0.7420774955720686 5 YJR091C Member of the Puf family of RNA-binding proteins; interacts with mRNAs encoding membrane-associated proteins; involved in localizing the Arp2/3 complex to mitochondria; overexpression causes increased sensitivity to benomyl; JSN1 has a paralog, PUF2, that arose from the whole genome duplication [Source:SGD;Acc:S000003851] 0.7277249150014757 94 YBR170C Substrate-recruiting cofactor of the Cdc48p-Npl4p-Ufd1p segregase; assists Cdc48p in the dislocation of misfolded, polyubiquitinated ERAD substrates that are subsequently delivered to the proteasome for degradation; also involved in the regulated destruction of resident ER membrane proteins, such as HMG-CoA reductase (Hmg1/2p) and cytoplasmic proteins (Fbp1p); role in mobilizing membrane bound transcription factors by regulated ubiquitin/proteasome-dependent processing (RUP) [Source:SGD;Acc:S000000374] 0.725455259881785 24 YHL038C Required for splicing of the group I intron bI5 of the COB pre-mRNA; nuclear-encoded mitochondrial protein that binds to the RNA to promote splicing; also involved in but not essential for splicing of the COB bI2 intron and the intron in the 21S rRNA gene [Source:SGD;Acc:S000001030] 0.7243772829976333 96 YOR256C Transferrin receptor-like protein; functions with Tre1p to regulate ubiquitination and vacuolar degradation of the metal transporter Smf1p; inviability of null mutant in systematic studies is due to proximity to CDC31; TRE2 has a paralog, TRE1, that arose from the whole genome duplication [Source:SGD;Acc:S000005782] 0.7243007653184526 95 YDR335W Karyopherin; involved in nuclear import and export of proteins, including import of replication protein A and export of Far1p and transcription factors Swi5p, Swi6p, Msn2p, and Pho4p; required for re-export of mature tRNAs after their retrograde import from the cytoplasm; exportin-5 homolog [Source:SGD;Acc:S000002743] 0.7213890603658258 73 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] 0.7185169202167437 48 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] 0.7150064895837985 20 YOL060C Protein required for normal mitochondrial morphology; has similarity to hemolysins [Source:SGD;Acc:S000005421] 0.712410349181063 21 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] 0.7099693744160611 24 YPL023C Protein with MTHFR activity in vitro; null mutant has no phenotype and is prototrophic for methionine; MET13 encodes major isozyme of methylenetetrahydrofolate reductase (MTHFR) [Source:SGD;Acc:S000005944] 0.7053602536386266 31 YEL025C Putative protein of unknown function; green fluorescent protein (GFP)-fusion protein localizes to both the cytoplasm and the nucleus [Source:SGD;Acc:S000000751] 0.7037649977814832 81 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] 0.6918675001771192 54 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] 0.69080261243635 98 YML013W Bridging factor involved in ER-associated protein degradation (ERAD); bridges the cytosolic Cdc48p-Npl1p-Ufd1p ATPase complex and the membrane associated Ssm4p and Hrd1p ubiquitin ligase complexes; contains a UBX (ubiquitin regulatory X) domain and a ubiquitin-associated (UBA) domain; redistributes from the ER to lipid droplets during the diauxic shift and stationary phase; required for the maintenance of lipid homeostasis [Source:SGD;Acc:S000004475] 0.6893538870389718 60 YMR054W Subunit a of the vacuolar-ATPase V0 domain; one of two isoforms (Stv1p and Vph1p); Stv1p is located in V-ATPase complexes of the Golgi and endosomes while Vph1p is located in V-ATPase complexes of the vacuole [Source:SGD;Acc:S000004658] 0.6892396652221591 61 YPL176C Transferrin receptor-like protein; plasma membrane protein that binds Bsd2p and regulates ubiquitination and vacuolar degradation of the metal transporter Smf1p; functionally redundant with Tre2p; TRE1 has a paralog, TRE2, that arose from the whole genome duplication [Source:SGD;Acc:S000006097] 0.6888553126860575 64 YFL009W F-box protein required for both the G1/S and G2/M phase transitions; modular substrate specificity factor which associates with core SCF (Cdc53p, Skp1p and Hrt1p/Rbx1p) to form the SCFCdc4 complex; SCFCdc4 acts as a ubiquitin-protein ligase directing ubiquitination of cyclin-dependent kinase (CDK) phosphorylated substrates, such as: Sic1p, Far1p, Cdc6p, Clb6p, and Cln3p [Source:SGD;Acc:S000001885] 0.6871471907579563 67 YJL054W Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane [Source:SGD;Acc:S000003590] 0.6866951763566563 68 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] 0.6856630644049064 69 YLR234W DNA Topoisomerase III; conserved protein that functions in a complex with Sgs1p and Rmi1p to relax single-stranded negatively-supercoiled DNA preferentially; DNA catenation/decatenation activity is stimulated by RPA and Sgs1p-Top3p-Rmi1p; involved in telomere stability and regulation of mitotic recombination [Source:SGD;Acc:S000004224] 0.6849952127532941 70 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] 0.6846402564355085 71 YER017C Mitochondrial inner membrane m-AAA protease component; mediates degradation of misfolded or unassembled proteins; also required for correct assembly of mitochondrial enzyme complexes; involved in cytoplasmic mRNA translation and aging; expression of human homolog AFG3L2 can complement yeast yta12 afg3 double mutant [Source:SGD;Acc:S000000819] 0.6834794602619109 76 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] 0.6821749135067937 80 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] 0.6742559247553176 99 YPL190C RNA-binding protein, subunit of Nrd1 complex (Nrd1p-Nab3p-Sen1p); complex interacts with exosome to mediate 3'-end formation of some mRNAs, snRNAs, snoRNAs, and CUTs; required for termination of non-poly(A) transcripts and efficient splicing; Nrd1-Nab3 pathway appears to have a role in rapid suppression of some genes when cells are shifted to poor growth conditions, indicating role for Nrd1-Nab3 in regulating cellular response to nutrient availability [Source:SGD;Acc:S000006111] 0.6741911641808375 100