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OM14 | Mitochondrial outer membrane receptor for cytosolic ribosomes; integral protein of the outer membrane that interacts with the nascent chain-associated complex (NAC) bound to ribosomes, contributing to co-translational mitochondrial import; interacts with porin (Por1p) and Om45p; abundance is decreased in cells grown in glucose relative to other carbon sources. (134 aa) | ||||
CDC48 | Cell division control protein 48; AAA ATPase; subunit of polyUb-selective segregase complex involved in ERAD, INM-associated degradation (INMAD), mitotic spindle disassembly, macroautophagy, PMN, ribosome-associated degradation, ribophagy, homotypic ER membrane fusion, SCF complex disassembly, cell wall integrity during heat stress, and telomerase regulation; mobilizes membrane-anchored transcription factors by regulated Ub/proteasome-dependent processing (RUP); human ortholog VCP complements a cdc48 mutant. (835 aa) | ||||
VMS1 | Protein VMS1; Component of a Cdc48p-complex involved in protein quality control; exhibits cytosolic and ER-membrane localization, with Cdc48p, during normal growth, and contributes to ER-associated degradation (ERAD) of specific substrates at a step after their ubiquitination; forms a mitochondrially-associated complex with Cdc48p and Npl4p under oxidative stress that is required for ubiquitin-mediated mitochondria-associated protein degradation (MAD); conserved in C. elegans and humans; Belongs to the ANKZF1/VMS1 family. (632 aa) | ||||
TPI1 | Triose phosphate isomerase, abundant glycolytic enzyme; mRNA half-life is regulated by iron availability; transcription is controlled by activators Reb1p, Gcr1p, and Rap1p through binding sites in the 5' non-coding region; inhibition of Tpi1p activity by PEP (phosphoenolpyruvate) stimulates redox metabolism in respiring cells; E104D mutation in human homolog TPI1 causes a rare autosomal disease; human TPI1 can complement yeast null mutant. (248 aa) | ||||
DOA4 | Ubiquitin hydrolase; deubiquitinates intralumenal vesicle (ILVs) cargo proteins; required for recycling ubiquitin from proteasome-bound ubiquitinated intermediates, acts at the late endosome/prevacuolar compartment to recover ubiquitin from ubiquitinated membrane proteins destined for the vacuole; DOA4 has a paralog, UBP5, that arose from the whole genome duplication; Belongs to the peptidase C19 family. (926 aa) | ||||
KGD2 | 2-oxoglutarate dehydrogenase E2 component (dihydrolipoamide succinyltransferase); Dihydrolipoyl transsuccinylase; component of the mitochondrial alpha-ketoglutarate dehydrogenase complex, which catalyzes the oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA in the TCA cycle; phosphorylated. (463 aa) | ||||
SDH4 | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial; Membrane anchor subunit of succinate dehydrogenase (SDH); involved in coupling the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; has similarity to human SDH subunit D (SDHD), which is implicated in paraganglioma. (181 aa) | ||||
MFB1 | Mitochondria-associated F-box protein; involved in maintenance of normal mitochondrial morphology; interacts with Skp1p through the F-box motif; preferentially localizes to the mother cell during budding. (465 aa) | ||||
TIM11 | Subunit e of mitochondrial F1F0-ATPase; ATPase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; essential for the dimeric and oligomeric state of ATP synthase, which in turn determines the shape of inner membrane cristae. (96 aa) | ||||
GGA1 | ADP-ribosylation factor-binding protein GGA1; Golgi-localized protein with homology to gamma-adaptin; interacts with and regulates Arf1p and Arf2p in a GTP-dependent manner in order to facilitate traffic through the late Golgi; GGA1 has a paralog, GGA2, that arose from the whole genome duplication. (557 aa) | ||||
TIM9 | Mitochondrial import inner membrane translocase subunit TIM9; Essential protein of the mitochondrial intermembrane space; forms a complex with Tim10p (TIM10 complex) that delivers hydrophobic proteins to the TIM22 complex for insertion into the inner membrane. (87 aa) | ||||
AFG3 | Mitochondrial respiratory chain complexes assembly protein AFG3; 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; In the N-terminal section; belongs to the AAA ATPase family. (761 aa) | ||||
RSP5 | NEDD4 family E3 ubiquitin ligase; regulates processes including: MVB sorting, the heat shock response, transcription, endocytosis and ribosome stability; ubiquitinates Sec23p, Sna3p, Ste4p, Nfi1p, Rpo21p and Sem1p; autoubiquitinates; deubiquitinated by Ubp2p; regulated by SUMO ligase Siz1p, in turn regulates Siz1p SUMO ligase activity; required for efficient Golgi-to-ER trafficking in COPI mutants; mutant tolerates aneuploidy; human homolog implicated in Liddle syndrome; Belongs to the RSP5/NEDD4 family. (809 aa) | ||||
MDJ1 | DnaJ homolog 1, mitochondrial; Co-chaperone that stimulates HSP70 protein Ssc1p ATPase activity; involved in protein folding/refolding in the mitochodrial matrix; required for proteolysis of misfolded proteins; member of the HSP40 (DnaJ) family of chaperones. (511 aa) | ||||
LPD1 | Dihydrolipoyl dehydrogenase, mitochondrial; Dihydrolipoamide dehydrogenase; the lipoamide dehydrogenase component (E3) of the pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase multi-enzyme complexes; PDH complex is concentrated in spots within the mitochondrial matrix, often near the ERMES complex and near peroxisomes; LPD1 has a paralog, IRC15, that arose from the whole genome duplication; Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family. (499 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) | ||||
COX4 | Subunit IV of cytochrome c oxidase; the terminal member of the mitochondrial inner membrane electron transport chain; precursor N-terminal 25 residues are cleaved during mitochondrial import; phosphorylated; spermidine enhances translation. (155 aa) | ||||
MSP1 | Protein MSP1; Highly-conserved N-terminally anchored AAA-ATPase; distributed in the mitochondrial outer membrane and peroxisomes; involved in mitochondrial protein sorting; functions as an extraction engine in local organelle surveillance to remove and initiate degradation of mistargeted proteins, ensuring fidelity of organelle-specific localization of tail-anchored proteins; contains an N-terminal transmembrane domain and C-terminal cytoplasmic ATPase domain. (362 aa) | ||||
TOM20 | Mitochondrial import receptor subunit TOM20; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; acts as a receptor for incoming precursor proteins. (183 aa) | ||||
SHY1 | Cytochrome oxidase assembly protein SHY1; Mitochondrial inner membrane protein required for complex IV assembly; associates with complex IV assembly intermediates and complex III/complex IV supercomplexes; similar to human SURF1 involved in Leigh Syndrome; complex IV is also known as cytochrome c oxidase. (389 aa) | ||||
PHB1 | Prohibitin-1; Subunit of the prohibitin complex (Phb1p-Phb2p); prohibitin is a 1.2 MDa ring-shaped inner mitochondrial membrane chaperone that stabilizes newly synthesized proteins; determinant of replicative life span; involved in mitochondrial segregation; prohibitin deficiency induces a mitochondrial unfolded protein response (mtUPR). (287 aa) | ||||
PRE9 | Alpha 3 subunit of the 20S proteasome; the only nonessential 20S subunit; may be replaced by the alpha 4 subunit (Pre6p) under stress conditions to create a more active proteasomal isoform; Belongs to the peptidase T1A family. (258 aa) | ||||
PHB2 | Prohibitin-2; Subunit of the prohibitin complex (Phb1p-Phb2p); prohibitin is a 1.2 MDa ring-shaped inner mitochondrial membrane chaperone that stabilizes newly synthesized proteins; determinant of replicative life span; involved in mitochondrial segregation; prohibitin deficiency induces a mitochondrial unfolded protein response (mtUPR). (310 aa) | ||||
TIM10 | Mitochondrial import inner membrane translocase subunit TIM10; Essential protein of the mitochondrial intermembrane space; forms a complex with Tim9p (TIM10 complex) that delivers hydrophobic proteins to the TIM22 complex for insertion into the inner membrane. (93 aa) | ||||
NCP1 | NADP-cytochrome P450 reductase; involved in ergosterol biosynthesis; associated and coordinately regulated with Erg11p; In the N-terminal section; belongs to the flavodoxin family. (691 aa) | ||||
COX6 | Subunit VI of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; expression is regulated by oxygen levels. (148 aa) | ||||
SAM35 | Component of the sorting and assembly machinery (SAM) complex; the SAM (or TOB) complex is located in the mitochondrial outer membrane; the complex binds precursors of beta-barrel proteins and facilitates their insertion into the outer membrane. (329 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) | ||||
SSM4 | ERAD-associated E3 ubiquitin-protein ligase DOA10; Membrane-embedded ubiquitin-protein ligase; ER and inner nuclear membrane localized RING-CH domain E3 ligase involved in ER-associated protein degradation (ERAD); targets misfolded cytosolic/nucleoplasmic domains of soluble and membrane embedded proteins (ERAD-C) and a transmembrane domain containing substrate (ERAD-M), Sbh2p; C-terminal element (CTE), conserved in human ortholog MARCH10/TEB4, determines substrate selectivity. (1319 aa) | ||||
POR2 | Mitochondrial outer membrane protein porin 2; Putative mitochondrial porin (voltage-dependent anion channel); not required for mitochondrial membrane permeability or mitochondrial osmotic stability; POR2 has a paralog, POR1, that arose from the whole genome duplication. (281 aa) | ||||
KGD1 | 2-oxoglutarate dehydrogenase, mitochondrial; Subunit of the mitochondrial alpha-ketoglutarate dehydrogenase complex; catalyzes a key step in the tricarboxylic acid (TCA) cycle, the oxidative decarboxylation of alpha-ketoglutarate to form succinyl-CoA. (1014 aa) | ||||
OM45 | Mitochondrial outer membrane protein of unknown function; major constituent of the outer membrane, extending into the intermembrane space; interacts with porin (Por1p) and with Om14p; imported via the presequence pathway involving the TOM and TIM23 complexes, then assembled in the outer membrane by Mim1p; protein abundance increases in response to DNA replication stress. (393 aa) | ||||
COA1 | Cytochrome c oxidase assembly factor 1; Mitochondrial inner membrane protein; required for assembly of the cytochrome c oxidase complex (complex IV); interacts with complex IV assembly factor Shy1p during the early stages of assembly. (197 aa) | ||||
AIM24 | Altered inheritance of mitochondria protein 24, mitochondrial; Protein with a role in determining mitochondrial architecture; inner membrane protein that interacts physically and genetically with the MICOS complex and is required for its integrity. (394 aa) | ||||
ATP2 | Beta subunit of the F1 sector of mitochondrial F1F0 ATP synthase; which is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated; Belongs to the ATPase alpha/beta chains family. (511 aa) | ||||
SDH3 | Subunit of succinate dehydrogenase and of TIM22 translocase; functions as cytochrome b subunit of succinate dehydrogenase, which couples oxidation of succinate to transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; also required for mitochondrial inner membrane protein import as part of the TIM22 complex; SDH3 has a paralog, SHH3, that arose from the whole genome duplication. (198 aa) | ||||
PAM17 | Presequence translocated-associated motor subunit PAM17, mitochondrial; Constituent of the TIM23 complex; proposed alternatively to be a component of the import motor (PAM complex) or to interact with and modulate the core TIM23 (Translocase of the Inner mitochondrial Membrane) complex; protein abundance increases in response to DNA replication stress; Belongs to the PAM17 family. (197 aa) | ||||
RLP24 | Essential protein required for ribosomal large subunit biogenesis; associated with pre-60S ribosomal subunits; stimulates the ATPase activity of Afg2p, which is required for release of Rlp24p from the pre-60S particle; has similarity to Rpl24Ap and Rpl24Bp. (199 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) | ||||
HSP60 | Heat shock protein 60, mitochondrial; Tetradecameric mitochondrial chaperonin; required for ATP-dependent folding of precursor polypeptides and complex assembly; prevents aggregation and mediates protein refolding after heat shock; role in mtDNA transmission; phosphorylated. (572 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) | ||||
MDM30 | Mitochondrial distribution and morphology protein 30; F-box component of an SCF ubiquitin protein ligase complex; associates with and is required for Fzo1p ubiquitination and for mitochondria fusion; stimulates nuclear export of specific mRNAs; promotes ubiquitin-mediated degradation of Gal4p in some strains. (598 aa) | ||||
SAM37 | Component of the Sorting and Assembly Machinery (SAM) complex; the SAM (or TOB) complex is located in the mitochondrial outer membrane; binds precursors of beta-barrel proteins and facilitates their outer membrane insertion; contributes to SAM complex stability. (327 aa) | ||||
UBX2 | UBX domain-containing protein 2; 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. (584 aa) | ||||
QCR2 | Subunit 2 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; phosphorylated; transcription is regulated by Hap1p, Hap2p/Hap3p, and heme. (368 aa) | ||||
TOM5 | Mitochondrial import receptor subunit TOM5; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import of all mitochondrially directed proteins; involved in transfer of precursors from the Tom70p and Tom20p receptors to the Tom40p pore; Belongs to the Tom5 family. (50 aa) | ||||
ATP20 | Subunit g of the mitochondrial F1F0 ATP synthase; reversibly phosphorylated on two residues; unphosphorylated form is required for dimerization of the ATP synthase complex, which in turn determines oligomerization of the complex and the shape of inner membrane cristae. (115 aa) | ||||
YPL191C | Ubiquitin carboxyl-terminal hydrolase MIY1; Protein of unknown function; diploid deletion strain exhibits high budding index; green fluorescent protein (GFP)-fusion protein localizes to the cytoplasm; YPL191C has a paralog, YGL082W, that arose from the whole genome duplication; Belongs to the MINDY deubiquitinase family. FAM63 subfamily. (360 aa) | ||||
NOG1 | Nucleolar GTP-binding protein 1; Putative GTPase; associates with free 60S ribosomal subunits in the nucleolus and is required for 60S ribosomal subunit biogenesis; constituent of 66S pre-ribosomal particles; member of the ODN family of nucleolar G-proteins. (647 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) | ||||
UBP16 | Ubiquitin carboxyl-terminal hydrolase 16; Deubiquitinating enzyme anchored to the outer mitochondrial membrane; probably not important for general mitochondrial functioning, but may perform a more specialized function at mitochondria; Belongs to the peptidase C19 family. (499 aa) | ||||
HRD1 | ERAD-associated E3 ubiquitin-protein ligase HRD1; Ubiquitin-protein ligase involved in ER-associated degradation (ERAD) of misfolded proteins; upon autoubiquitination triggers retrotranslocation of misfolded proteins to cytosol for degradation; genetically linked to the unfolded protein response (UPR); regulated through association with Hrd3p; contains an H2 ring finger; likely plays a general role in targeting proteins that persistently associate with and potentially obstruct the ER-localized translocon; Belongs to the HRD1 family. (551 aa) | ||||
MDJ2 | Mitochondrial DnaJ homolog 2; Constituent of the mitochondrial import motor; associated with the presequence translocase; function overlaps with that of Pam18p; stimulates the ATPase activity of Ssc1p to drive mitochondrial import; contains a J domain. (146 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) | ||||
TOM22 | Mitochondrial import receptor subunit TOM22; Component of the TOM (Translocase of Outer Membrane) complex; responsible for initial import of mitochondrially directed proteins; mediates interaction between TOM and TIM complexes and acts as a receptor for precursor proteins; Belongs to the Tom22 family. (152 aa) | ||||
TOM70 | Mitochondrial import receptor subunit TOM70; 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. (617 aa) | ||||
SAM50 | Component of the Sorting and Assembly Machinery (SAM) complex; the SAM (or TOB) complex is located in the mitochondrial outer membrane; the complex binds precursors of beta-barrel proteins and facilitates their outer membrane insertion; homologous to bacterial Omp85. (484 aa) | ||||
ATM1 | Iron-sulfur clusters transporter ATM1, mitochondrial; Mitochondrial inner membrane ATP-binding cassette (ABC) transporter; exports mitochondrially synthesized precursors of iron-sulfur (Fe/S) clusters to the cytosol; human homolog ABCB7 can complement yeast null mutant; Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily. (690 aa) | ||||
DSK2 | Nuclear-enriched ubiquitin-like polyubiquitin-binding protein; required for spindle pole body (SPB) duplication and for transit through the G2/M phase of the cell cycle; involved in proteolysis; interacts with the proteasome; protein abundance increases in response to DNA replication stress. (373 aa) | ||||
PEP5 | Histone E3 ligase, component of CORVET membrane tethering complex; peripheral vacuolar membrane protein required for protein trafficking and vacuole biogenesis; interacts with Pep7p; involved in ubiquitination and degradation of excess histones; Belongs to the VPS11 family. (1029 aa) | ||||
TOM40 | Mitochondrial import receptor subunit TOM40; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; constitutes the core element of the protein conducting pore; pre-Tom40p is phosphorylated by PKA, which impairs its import into mitochondria under non-respiratory conditions. (387 aa) | ||||
YTA12 | Mitochondrial respiratory chain complexes assembly protein YTA12; 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; In the N-terminal section; belongs to the AAA ATPase family. (825 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) | ||||
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) | ||||
MDM10 | Mitochondrial distribution and morphology protein 10; Subunit of both the ERMES and the SAM complex; component of ERMES complex which acts as a molecular tether between the mitochondria and the ER, necessary for efficient phospholipid exchange between organelles and for mitophagy; SAM/TOB complex component that functions in the assembly of outer membrane beta-barrel proteins; involved in mitochondrial inheritance and morphology; ERMES complex is often co-localized with peroxisomes and concentrated areas of pyruvate dehydrogenase; Belongs to the MDM10 family. (493 aa) | ||||
PTH2 | Peptidyl-tRNA hydrolase 2; One of two mitochondrially-localized peptidyl-tRNA hydrolases; negatively regulates the ubiquitin-proteasome pathway via interactions with ubiquitin-like ubiquitin-associated proteins; dispensable for cell growth; see also PTH1. (208 aa) | ||||
ATP1 | Alpha subunit of the F1 sector of mitochondrial F1F0 ATP synthase; which is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated; N-terminally propionylated in vivo; Belongs to the ATPase alpha/beta chains family. (545 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) |