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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) | ||||
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) | ||||
MIX23 | Mitochondrial intermembrane space protein of unknown function; imported via the MIA import machinery; contains an unusual twin cysteine motif (CX13C CX14C). (196 aa) | ||||
TIM12 | Mitochondrial import inner membrane translocase subunit TIM12; Essential protein of the inner mitochondrial membrane; peripherally localized; component of the TIM22 complex, which is a twin-pore translocase that mediates insertion of numerous multispanning inner membrane proteins. (109 aa) | ||||
TRX3 | Mitochondrial thioredoxin; highly conserved oxidoreductase required to maintain the redox homeostasis of the cell, forms the mitochondrial thioredoxin system with Trr2p, redox state is maintained by both Trr2p and Glr1p. (127 aa) | ||||
MRP10 | Mitochondrial ribosomal protein of the small subunit; contains twin cysteine-x9-cysteine motifs; oxidized by Mia40p during import into mitochondria. (95 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) | ||||
TRR1 | Cytoplasmic thioredoxin reductase; key regulatory enzyme that determines the redox state of the thioredoxin system, which acts as a disulfide reductase system and protects cells against both oxidative and reductive stress; protein abundance increases in response to DNA replication stress; TRR1 has a paralog, TRR2, that arose from the whole genome duplication; Belongs to the class-II pyridine nucleotide-disulfide oxidoreductase family. (319 aa) | ||||
CYM1 | Mitochondrial presequence protease; 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. (989 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) | ||||
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) | ||||
SOM1 | Protein SOM1, mitochondrial; Subunit of the mitochondrial inner membrane peptidase (IMP); IMP is required for maturation of mitochondrial proteins of the intermembrane space; Som1p facilitates cleavage of a subset of substrates; contains twin cysteine-x9-cysteine motifs. (74 aa) | ||||
ICP55 | Intermediate cleaving peptidase 55; Mitochondrial aminopeptidase; cleaves the N termini of at least 38 imported proteins after cleavage by the mitochondrial processing peptidase (MPP), thereby increasing their stability; member of the aminopeptidase P family. (511 aa) | ||||
ERV1 | Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation. (189 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) | ||||
TRX2 | Thioredoxin-2; Cytoplasmic thioredoxin isoenzyme; part of thioredoxin system which protects cells against oxidative and reductive stress; forms LMA1 complex with Pbi2p; acts as a cofactor for Tsa1p; required for ER-Golgi transport and vacuole inheritance; with Trx1p, facilitates mitochondrial import of small Tims Tim9p, Tim10p, Tim13p by maintaining them in reduced form; abundance increases under DNA replication stress; TRX2 has a paralog, TRX1, that arose from the whole genome duplication. (104 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) | ||||
MAS2 | Alpha subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins. (482 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) | ||||
HYR1 | Glutathione peroxidase-like peroxiredoxin HYR1; Thiol peroxidase; functions as a hydroperoxide receptor to sense intracellular hydroperoxide levels and transduce a redox signal to the Yap1p transcription factor; HYR1 has a paralog, GPX1, that arose from the whole genome duplication. (163 aa) | ||||
TIM54 | Mitochondrial import inner membrane translocase subunit TIM54; Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane. (478 aa) | ||||
TIM17 | Mitochondrial import inner membrane translocase subunit TIM17; Essential component of the TIM23 complex; with Tim23p, contributes to the architecture and function of the import channel; may link the import motor to the core Translocase of the Inner Mitochondrial membrane (TIM23 complex); Belongs to the Tim17/Tim22/Tim23 family. (158 aa) | ||||
SSC1 | Heat shock protein SSC1, mitochondrial; Hsp70 family ATPase; constituent of the import motor component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); involved in protein translocation and folding; subunit of SceI endonuclease; SSC1 has a paralog, ECM10, that arose from the whole genome duplication. (654 aa) | ||||
OSM1 | Fumarate reductase, catalyzes the reduction of fumarate to succinate; required for the reoxidation of intracellular NADH under anaerobic conditions; mutations cause osmotic sensitivity; has two translation start sites, one at the annotated start codon which produces an ER-targeted form required for anaerobic growth, and one at codon 32 which produces a mitochondrially-targeted form; OSM1 has a paralog, FRD1, that arose from the whole genome duplication; Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily. (501 aa) | ||||
TIM8 | Mitochondrial import inner membrane translocase subunit TIM8; Mitochondrial intermembrane space protein; forms a complex with Tim13p that delivers a subset of hydrophobic proteins to the TIM22 complex for inner membrane insertion; homolog of human TIMM8A, implicated in Mohr-Tranebjaerg syndrome, also known as deafness-dystonia-optic neuronopathy (DDON) syndrome; human TIMM8A can complement yeast null mutant. (87 aa) | ||||
CYT2 | Cytochrome c1 heme lyase; involved in maturation of cytochrome c1, which is a subunit of the mitochondrial ubiquinol-cytochrome-c reductase; links heme covalently to apocytochrome c1; human homolog HCCS can complement yeast cyt2 null mutant. (224 aa) | ||||
OCT1 | Mitochondrial intermediate peptidase; cleaves destabilizing N-terminal residues of a subset of proteins upon import, after their cleavage by mitochondrial processing peptidase (Mas1p-Mas2p); may contribute to mitochondrial iron homeostasis. (772 aa) | ||||
MIA40 | 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. (403 aa) | ||||
CCP1 | Mitochondrial cytochrome-c peroxidase; degrades reactive oxygen species in mitochondria, involved in the response to oxidative stress. (361 aa) | ||||
COX17 | Copper metallochaperone that transfers copper to Sco1p and Cox11p; eventual delivery to cytochrome c oxidase; contains twin cysteine-x9-cysteine motifs; interacts with the MICOS complex, and interaction is promoted by copper ions; human homolog COX17 partially complements yeast null mutant. (69 aa) | ||||
TRX1 | Thioredoxin-1; Cytoplasmic thioredoxin isoenzyme; part of thioredoxin system which protects cells against oxidative and reductive stress; forms LMA1 complex with Pbi2p; acts as a cofactor for Tsa1p; required for ER-Golgi transport and vacuole inheritance; with Trx2p, facilitates mitochondrial import of small Tims Tim9p, Tim10p, Tim13p by maintaining them in reduced form; abundance increases iunder DNA replication stress; TRX1 has a paralog, TRX2, that arose from the whole genome duplication. (103 aa) | ||||
MIM2 | Mitochondrial protein required for outer membrane protein import; involved in import of the subset of proteins with multiple alpha-helical transmembrane segments, including Ugo1p, Tom20p, and Fzo1p; component of a large protein complex in the outer membrane that includes Mim1p; not essential in W303 strain background; Belongs to the MIM2 family. (87 aa) | ||||
MAS1 | Beta subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins; Belongs to the peptidase M16 family. (462 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) | ||||
STE23 | A-factor-processing enzyme; Metalloprotease; involved in N-terminal processing of pro-a-factor to mature form; expressed in both haploids and diploids; one of two yeast homologs of human insulin-degrading enzyme (hIDE); homolog Axl1p is also involved in processing of pro-a-factor; Belongs to the peptidase M16 family. (1027 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) | ||||
HSC82 | ATP-dependent molecular chaperone HSC82; Cytoplasmic chaperone of the Hsp90 family; plays a role in determining prion variants; redundant in function and nearly identical with Hsp82p, and together they are essential; expressed constitutively at 10-fold higher basal levels than HSP82 and induced 2-3 fold by heat shock; contains two acid-rich unstructured regions that promote the solubility of chaperone-substrate complexes; HSC82 has a paralog, HSP82, that arose from the whole genome duplication. (705 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) | ||||
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) | ||||
YDJ1 | Mitochondrial protein import protein MAS5; Type I HSP40 co-chaperone; involved in regulation of HSP90 and HSP70 functions; acts as an adaptor that helps Rsp5p recognize cytosolic misfolded proteins for ubiquitination after heat shock; critical for determining cell size at Start as a function of growth rate; involved in protein translocation across membranes; member of the DnaJ family; chimeric protein in which human p58IPK J domain replaces yeast Ydj1p J domain can complement yeast ydj1 mutant. (409 aa) | ||||
TOM7 | Mitochondrial import receptor subunit TOM7; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; promotes assembly and stability of the TOM complex; Belongs to the Tom7 family. (60 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) | ||||
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) | ||||
ATP23 | Putative metalloprotease of the mitochondrial inner membrane; required for processing of Atp6p; has an additional role in assembly of the F0 sector of the F1F0 ATP synthase complex; substrate of the Mia40p-Erv1p disulfide relay system, and folding is assisted by Mia40p; Belongs to the peptidase M76 family. (270 aa) | ||||
MIM1 | Mitochondrial protein required for outer membrane protein import; cooperates with Tom70p to import the subset of proteins with multiple alpha-helical transmembrane segments, including Ugo1p, Tom20p, and others; present in a complex with Mim2p in the outer membrane that may create a local environment to facilitate membrane insertion of substrate proteins; also has a role in assembly of Tom20p into the TOM complex; Belongs to the MIM1 family. (113 aa) | ||||
HSP10 | 10 kDa heat shock protein, mitochondrial; Mitochondrial matrix co-chaperonin; inhibits the ATPase activity of Hsp60p, a mitochondrial chaperonin; involved in protein folding and sorting in the mitochondria; 10 kD heat shock protein with similarity to E. coli groES. (106 aa) | ||||
TOM6 | Mitochondrial import receptor subunit TOM6; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; promotes assembly and stability of the TOM complex. (61 aa) | ||||
CYT1 | Cytochrome c1, heme protein, mitochondrial; Cytochrome c1; component of the mitochondrial respiratory chain; expression is regulated by the heme-activated, glucose-repressed Hap2p/3p/4p/5p CCAAT-binding complex. (309 aa) | ||||
DFR1 | Dihydrofolate reductase involved in tetrahydrofolate biosynthesis; required for respiratory metabolism; mutation is functionally complemented by human DHFR. (211 aa) | ||||
TIM18 | Mitochondrial import inner membrane translocase subunit TIM18; Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane; may mediate assembly or stability of the complex; Belongs to the CybS family. (192 aa) | ||||
TIM50 | 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. (476 aa) | ||||
MGR2 | Protein MGR2; Subunit of the TIM23 translocase complex; acts to couple Tim21p with the core Tim23 translocase; provides quality control for insertion of membrane proteins by regulating their release into the inner membrane by the TIM23 complex; absolutely required for mitochondrial import of presequence-containing proteins at elevated temperature; required for viability of cells lacking the mitochondrial genome (petite-negative phenotype); Belongs to the MGR2 family. (113 aa) | ||||
HSP82 | ATP-dependent molecular chaperone HSP82; Hsp90 chaperone; redundant in function with Hsc82p; required for pheromone signaling, negative regulation of Hsf1p; docks with Tom70p for mitochondrial preprotein delivery; promotes telomerase DNA binding, nucleotide addition; protein abundance increases in response to DNA replication stress; contains two acid-rich unstructured regions that promote solubility of chaperone-substrate complexes; HSP82 has a paralog, HSC82, that arose from the whole genome duplication. (709 aa) | ||||
YME1 | 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; In the C-terminal section; belongs to the peptidase M41 family. (747 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) |