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PET111 | Protein PET111, mitochondrial; Mitochondrial translational activator specific for the COX2 mRNA; located in the mitochondrial inner membrane. (800 aa) | ||||
NAM2 | Leucine--tRNA ligase, mitochondrial; 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; Belongs to the class-I aminoacyl-tRNA synthetase family. (894 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) | ||||
PET309 | Pentatricopeptide repeat-containing protein PET309, mitochondrial; Specific translational activator for the COX1 mRNA; binds to the COX1 mRNA; also influences stability of intron-containing COX1 primary transcripts; localizes to the mitochondrial inner membrane; contains 12 pentatricopeptide repeats (PPRs). (965 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) | ||||
RSM22 | Mitochondrial ribosomal protein of the small subunit; also predicted to be an S-adenosylmethionine-dependent RNA methyltransferase. (628 aa) | ||||
SOD1 | Cytosolic copper-zinc superoxide dismutase; detoxifies superoxide; stabilizes Yck1p and Yck2p kinases in glucose to repress respiration; phosphorylated by Dun1p, enters nucleus under oxidative stress to promote transcription of stress response genes; human ortholog SOD1 implicated in ALS complements a null allele; abundance increases under DNA replication stress and during exposure to boric acid; localization to mitochondrial intermembrane space is modulated by MICOS complex; Belongs to the Cu-Zn superoxide dismutase family. (154 aa) | ||||
COX23 | Cytochrome c oxidase-assembly factor COX23, mitochondrial; Protein that functions in mitochondrial copper homeostasis; mitochondrial intermembrane space protein; essential for functional cytochrome oxidase expression; homologous to Cox17p; contains twin cysteine-x9-cysteine motifs. (151 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) | ||||
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) | ||||
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) | ||||
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) | ||||
YGR021W | Probable transcriptional regulatory protein HAH1; Putative protein of unknown function; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies; Belongs to the TACO1 family. (290 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) | ||||
COX15 | Cytochrome c oxidase assembly protein COX15; Protein required for the hydroxylation of heme O to form heme A; heme A is an essential prosthetic group for cytochrome c oxidase; Belongs to the COX15/CtaA family. (486 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) | ||||
ARH1 | Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family. (493 aa) | ||||
MSS116 | ATP-dependent RNA helicase MSS116, mitochondrial; Mitochondrial transcription elongation factor; DEAD-box protein; required for efficient splicing of mitochondrial Group I and II introns; non-polar RNA helicase that also facilities strand annealing; promotes RNA folding by stabilizing an early assembly intermediate. (664 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) | ||||
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) | ||||
MTF2 | Mitochondrial transcription factor 2; Mitochondrial protein that interacts with mitochondrial RNA polymerase; interacts with an N-terminal region of mitochondrial RNA polymerase (Rpo41p) and couples RNA processing and translation to transcription. (440 aa) | ||||
ATX1 | Metal homeostasis factor ATX1; Cytosolic copper metallochaperone; transports copper to the secretory vesicle copper transporter Ccc2p for eventual insertion into Fet3p, which is a multicopper oxidase required for high-affinity iron uptake; human homolog ATOX1 can complement yeast atx1 mutant; overexpression of human ATOX1 suppresses lysine auxotrophy of the yeast sod1 null mutant, as does overexpression of yeast ATX1. (73 aa) | ||||
PET494 | Mitochondrial translational activator specific for the COX3 mRNA; acts together with Pet54p and Pet122p; located in the mitochondrial inner membrane. (489 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) | ||||
MNE1 | Protein involved in splicing Group I aI5-beta intron from COX1 mRNA; mitochondrial matrix protein. (663 aa) | ||||
SUV3 | 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. (737 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) | ||||
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) | ||||
COA2 | Cytochrome oxidase assembly factor; null mutation results in respiratory deficiency with specific loss of cytochrome oxidase activity; functions downstream of assembly factors Mss51p and Coa1p and interacts with assembly factor Shy1p; Belongs to the COA2 family. (68 aa) | ||||
YAH1 | Adrenodoxin homolog, mitochondrial; Ferredoxin of the mitochondrial matrix; required for formation of cellular iron-sulfur proteins; involved in heme A biosynthesis; human homolog FDX1L can complement yeast by allowing growth during down-regulation of yeast YAH1; Belongs to the adrenodoxin/putidaredoxin family. (172 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) | ||||
AI5_BETA | Intron-encoded DNA endonuclease aI5 beta; Protein of unknown function; encoded within an intron of the mitochondrial COX1 gene; translational initiation codon is predicted to be ATA rather than ATG. (354 aa) | ||||
AI1 | Putative COX1/OXI3 intron 1 protein; Reverse transcriptase required for splicing of the COX1 pre-mRNA; encoded by a mobile group II intron within the mitochondrial COX1 gene. (834 aa) | ||||
AI2 | Putative COX1/OXI3 intron 2 protein; Reverse transcriptase required for splicing of the COX1 pre-mRNA; encoded by a mobile group II intron within the mitochondrial COX1 gene. (854 aa) | ||||
AI4 | Truncated non-functional cytochrome oxidase 1; Endonuclease I-SceII; encoded by a mobile group I intron within the mitochondrial COX1 gene; intron is normally spliced by the BI4p maturase but AI4p can mutate to acquire the same maturase activity. (556 aa) | ||||
AI5_ALPHA | Truncated non-functional cytochrome oxidase 1; Endonuclease I-SceIV; involved in intron mobility; encoded by a mobile group I intron within the mitochondrial COX1 gene; In the N-terminal section; belongs to the heme-copper respiratory oxidase family. (630 aa) | ||||
MBA1 | Membrane-associated mitochondrial ribosome receptor; forms a complex with Mdm38p that may facilitate recruitment of mRNA-specific translational activators to ribosomes; possible role in protein export from the matrix to inner membrane. (278 aa) | ||||
SCO2 | Protein anchored to mitochondrial inner membrane; may have a redundant function with Sco1p in delivery of copper to cytochrome c oxidase; interacts with Cox2p; SCO2 has a paralog, SCO1, that arose from the whole genome duplication. (301 aa) | ||||
CMC2 | COX assembly mitochondrial protein 2; Protein involved in respiratory chain complex assembly or maintenance; protein of the mitochondrial intermembrane space; contains twin Cx9C motifs that can form coiled coil-helix-coiled-coil helix fold. (109 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) |