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CIR2 | Probable electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; Putative ortholog of human ETF-dH; found in a large supramolecular complex with other mitochondrial dehydrogenases; may have a role in oxidative stress response; ETF-dH is also known as electron transfer flavoprotein dehydrogenase. (631 aa) | ||||
INA17 | Inner membrane assembly complex subunit 17; F1F0 ATPase synthase peripheral stalk assembly factor; subunit of the matrix-exposed inner mitochondrial membrane localized INA complex (Ina22p-Ina17p) involved in assembly of the F1F0 peripheral stalk; co-purifies with Ina22p and ATP synthase subunits; null mutant displays elevated frequency of mitochondrial genome loss and has a respiratory growth defect. (182 aa) | ||||
FMP30 | N-acyl-phosphatidylethanolamine-hydrolyzing phospholipase D, mitochondrial; Protein with a role in maintaining mitochondrial morphology; also involved in maintaining normal cardiolipin levels; mitochondrial inner membrane protein; proposed to be involved in N-acylethanolamine metabolism; related to mammalian N-acylPE-specific phospholipase D; Belongs to the NAPE-PLD family. (468 aa) | ||||
COX11 | Cytochrome c oxidase assembly protein COX11, mitochondrial; Protein required for delivery of copper to Cox1p; mitochondrial inner membrane protein; association with mitochondrial ribosomes suggests that copper delivery may occur during translation of Cox1p; Belongs to the COX11/CtaG family. (300 aa) | ||||
JID1 | J domain-containing protein 1; Probable Hsp40p co-chaperone; has a DnaJ-like domain and appears to be involved in ER-associated degradation of misfolded proteins containing a tightly folded cytoplasmic domain; inhibits replication of Brome mosaic virus in S. cerevisiae. (301 aa) | ||||
TIM23 | Essential component of the TIM23 complex; involved in protein import into mitochondrial matrix and inner membrane; with Tim17p, contributes to architecture and function of the import channel; TIM23 complex is short for the translocase of the inner mitochondrial membrane. (222 aa) | ||||
PSD1 | Phosphatidylserine decarboxylase of the mitochondrial inner membrane; converts phosphatidylserine to phosphatidylethanolamine; regulates mitochondrial fusion and morphology by affecting lipid mixing in the mitochondrial membrane and by influencing the ratio of long to short forms of Mgm1p; partly exposed to the mitochondrial intermembrane space; autocatalytically processed; Belongs to the phosphatidylserine decarboxylase family. PSD-B subfamily. Eukaryotic type I sub-subfamily. (500 aa) | ||||
MIC27 | Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; forms a subcomplex with Mic10p and Mic12p whose assembly and stability requires cardiolipin; Belongs to the apolipoprotein O/MICOS complex subunit Mic27 family. (234 aa) | ||||
YME2 | Integral inner mitochondrial membrane protein; role in maintaining mitochondrial nucleoid structure and number; mutants exhibit an increased rate of mitochondrial DNA escape; shows some sequence similarity to exonucleases. (850 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) | ||||
RCF1 | Respiratory supercomplex factor 1, mitochondrial; Cytochrome c oxidase subunit; required for assembly of the Complex III-Complex IV supercomplex, and for assembly of Cox13p and Rcf2p into cytochrome c oxidase; similar to Rcf2p, and either Rcf1p or Rcf2p is required for late-stage assembly of the Cox12p and Cox13p subunits and for cytochrome c oxidase activity; required for growth under hypoxic conditions; member of the hypoxia induced gene family; C. elegans and human orthologs are functional in yeast. (159 aa) | ||||
QRI5 | Mitochondrial inner membrane protein; required for accumulation of spliced COX1 mRNA; may have an additional role in translation of COX1 mRNA. (111 aa) | ||||
FMP25 | Protein FMP25, mitochondrial; Protein required for assembly of respiratory complex III; mitochondrial inner membrane protein; required for an early step in assembly of respiratory complex III (cytochrome bc1 complex); mRNA is targeted to mitochondria. (583 aa) | ||||
MIC60 | Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; Mic60p is also involved in import of intermembrane space (IMS) proteins, probably by positioning Mia40p relative to the TOM complex to receive incoming proteins; ortholog of mammalian mitofilin. (540 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) | ||||
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) | ||||
MIR1 | Mitochondrial phosphate carrier; imports inorganic phosphate into mitochondria; functionally redundant with Pic2p but more abundant than Pic2p under normal conditions; phosphorylated; Belongs to the mitochondrial carrier (TC 2.A.29) family. (311 aa) | ||||
PET191 | Mitochondrial protein PET191; Protein required for assembly of cytochrome c oxidase; exists as an oligomer; described as both an integral mitochondrial inner membrane protein facing the intermembrane space (IMS) and as a soluble IMS protein; contains a twin Cx9C motif; imported into the IMS via the MIA import machinery. (108 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) | ||||
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) | ||||
COX16 | Mitochondrial inner membrane protein; required for assembly of cytochrome c oxidase. (118 aa) | ||||
INA22 | Inner membrane assembly complex subunit 22; F1F0 ATP synthase peripheral stalk assembly factor; subunit of the matrix-exposed inner mitochondrial membrane localized INA complex (Ina22p-Ina17p) involved in assembly of the F1F0 peripheral stalk; co-purifies with Aim43p, ATP synthase subunits, and cytochrome bc1 complex assembly factors; interacts with Arh1p, a mitochondrial oxidoreductase; deletion mutant has a respiratory growth defect. (216 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) | ||||
MPC2 | Highly conserved subunit of the mitochondrial pyruvate carrier (MPC); expressed during growth on fermentable carbon sources, and heterodimerizes with Mpc1p to form the fermentative isoform of MPC; MPC localizes to the mitochondrial inner membrane and mediates pyruvate uptake; MPC2 paralog, MPC3, heterodimerizes with Mpc1p to form the respiratory MPC isoform. (129 aa) | ||||
MPC3 | Highly conserved subunit of the mitochondrial pyruvate carrier (MPC); expressed during growth on nonfermentable carbon sources, and heterodimerizes with Mpc1p to form the respiratory isoform of MPC; MPC localizes to the mitochondrial inner membrane and mediates pyruvate uptake; MPC3 paralog, MPC2, heterodimerizes with Mpc1p to form the fermentative MPC isoform; protein abundance increases in response to DNA replication stress. (146 aa) | ||||
MIC26 | MICOS subunit MIC26; Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; Mic26p is a non-essential component of the complex. (233 aa) | ||||
TPC1 | Mitochondrial thiamine pyrophosphate carrier 1; Mitochondrial membrane transporter; mediates uptake of the essential cofactor thiamine pyrophosphate (ThPP) into mitochondria; expression appears to be regulated by carbon source; member of the mitochondrial carrier family. (314 aa) | ||||
COX18 | Cytochrome c oxidase assembly protein COX18, mitochondrial; Protein required for membrane insertion of C-terminus of Cox2p; mitochondrial integral inner membrane protein; interacts genetically and physically with Mss2p and Pnt1p; similar to S. cerevisiae Oxa1, N. crassa Oxa2p, and E. coli YidC; respiratory defect of the null mutant is functionally complemented by human COX18 carrying the N-terminal 54 amino acids of S. cerevisiae Cox18p; Belongs to the OXA1/ALB3/YidC family. (316 aa) | ||||
MPC1 | Highly conserved subunit of mitochondrial pyruvate carrier (MPC); MPC is a mitochondrial inner membrane complex that mediates pyruvate uptake and comprises Mpc1p and Mpc2p during fermentative growth, or Mcp1p and Mpc3p during respiratory growth; null mutant displays slow growth that is complemented by expression of human or mouse ortholog; mutation in human ortholog MPC1 is associated with lactic acidosis and hyperpyruvatemia. (130 aa) | ||||
MIC19 | Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; Mic19p is peripheral to the inner membrane and may connect Mic60p with the Mic10p-Mic12p-Mic27p subcomplex; both yeast and human Mic19p become oxidized, and oxidation may regulate MICOS. (170 aa) | ||||
OXA1 | Mitochondrial inner membrane insertase; mediates the insertion of both mitochondrial- and nuclear-encoded proteins from the matrix into the inner membrane; also has a role in insertion of carrier proteins into the inner membrane; acts as a voltage-gated ion channel, activated by substrate peptides; interacts with mitochondrial ribosomes; conserved from bacteria to animals. (402 aa) | ||||
PET122 | Protein PET122, mitochondrial; Mitochondrial translational activator specific for the COX3 mRNA; acts together with Pet54p and Pet494p; located in the mitochondrial inner membrane. (254 aa) | ||||
PIC2 | Mitochondrial copper and phosphate carrier; imports copper and inorganic phosphate into mitochondria; functionally redundant with Mir1p but less abundant than Mir1p under normal conditions; expression is induced at high temperature. (300 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) | ||||
HEM14 | Protoporphyrinogen oxidase; a mitochondrial enzyme that catalyzes the seventh step in the heme biosynthetic pathway, converting protoporphyrinogen IX to protoporphyrin IX; inhibited by diphenyl ether-type herbicides. (539 aa) | ||||
UGO1 | Outer membrane component of the mitochondrial fusion machinery; binds to Fzo1p and Mgm1p to link these two GTPases during mitochondrial fusion; involved in fusion of both the outer and inner membranes; facilitates dimerization of Fzo1p during fusion; import into the outer membrane is mediated by Tom70p and Mim1p; has similarity to carrier proteins but likely not a transporter; similar to human SLC25A46 implicated in optic atroprophy spectrum disorder. (502 aa) | ||||
YDR381C-A | Protein of unknown function; localized to the mitochondrial outer membrane. (114 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) | ||||
TIM22 | Mitochondrial import inner membrane translocase subunit TIM22; Essential core component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane; forms the channel through which proteins are imported; Belongs to the Tim17/Tim22/Tim23 family. (207 aa) | ||||
YDL183C | Uncharacterized protein YDL183C; Protein that may form an active mitochondrial KHE system; mitochondrial inner-membrane protein; non-essential gene; KHE system stands for K+/H+ exchanger system; To S.pombe SpAC23H3.12c. (320 aa) | ||||
MIC10 | Conserved component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; forms a subcomplex with Mic12p and Mic27p whose assembly and stability requires cardiolipin; homo-oligomers cause membrane bending; ortholog of human MINOS1. (97 aa) | ||||
MIC12 | Component of the MICOS complex; MICOS (formerly MINOS or MitOS) is a mitochondrial inner membrane complex that extends into the intermembrane space and has a role in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane; forms a subcomplex with Mic10p and Mic27p whose assembly and stability requires cardiolipin. (106 aa) | ||||
RCF2 | Respiratory supercomplex factor 2, mitochondrial; Cytochrome c oxidase subunit; has a role in assembly of respiratory supercomplexes; similar to Rcf1p, and either Rcf1p or Rcf2p is required for late-stage assembly of the Cox12p and Cox13p subunits and for cytochrome c oxidase activity; associates with the cytochrome c oxidase - cytochrome bc1 supercomplex; null mutant accumulates reactive oxygen species; member of the conserved hypoxia induced gene family; C. elegans homolog is functional in yeast. (224 aa) | ||||
COQ2 | 4-hydroxybenzoate polyprenyltransferase, mitochondrial; Para hydroxybenzoate polyprenyl transferase; catalyzes the second step in ubiquinone (coenzyme Q) biosynthesis; human COQ2, mutations in which are implicated in an increased risk of mutiple-system atrophy, can complement a yeast coq2 null mutant. (372 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) | ||||
PNT1 | Pentamidine resistance factor, mitochondrial; Mitochondrial integral inner membrane protein; involved in membrane insertion of C-terminus of Cox2p, interacts genetically and physically with Cox18p; deletion mutant sensitive to the anti-Pneumocystis carinii drug pentamidine. (423 aa) | ||||
FSF1 | Sideroflexin FSF1; Putative protein; predicted to be an alpha-isopropylmalate carrier; belongs to the sideroblastic-associated protein family; non-tagged protein is detected in purified mitochondria; likely to play a role in iron homeostasis. (327 aa) |