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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) | ||||
OLI1 | F0-ATP synthase subunit c (ATPase-associated proteolipid); encoded on the mitochondrial genome; mutation confers oligomycin resistance; expression is specifically dependent on the nuclear genes AEP1 and AEP2. (76 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) | ||||
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) | ||||
ATP3 | Gamma subunit of the F1 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis. (311 aa) | ||||
HSP26 | Small heat shock protein (sHSP) with chaperone activity; forms hollow, sphere-shaped oligomers that suppress unfolded proteins aggregation; long-lived protein that is preferentially retained in mother cells and forms cytoplasmic foci; oligomer activation requires heat-induced conformational change; also has mRNA binding activity. (214 aa) | ||||
ATP16 | Delta subunit of the central stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase 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. (160 aa) | ||||
ATP5 | Subunit 5 of the stator stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; homologous to bovine subunit OSCP (oligomycin sensitivity-conferring protein); phosphorylated; Belongs to the ATPase delta chain family. (212 aa) | ||||
RPT3 | ATPase of the 19S regulatory particle of the 26S proteasome; one of ATPases of the regulatory particle; involved in the degradation of ubiquitinated substrates; substrate of N-acetyltransferase B. (428 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) | ||||
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) | ||||
RPT6 | ATPase of the 19S regulatory particle of the 26S proteasome; one of six ATPases of the regulatory particle; involved in the degradation of ubiquitinated substrates; bound by ubiquitin-protein ligases Ubr1p and Ufd4p; localized mainly to the nucleus throughout the cell cycle; protein abundance increases in response to DNA replication stress. (405 aa) | ||||
PSD2 | Phosphatidylserine decarboxylase 2 alpha chain; Phosphatidylserine decarboxylase of the Golgi and vacuolar membranes; converts phosphatidylserine to phosphatidylethanolamine; controls vacuolar membrane phospholipid content by regulating phospholipids in compartments that will eventually give rise to the vacuole; loss of Psd2p causes a specific reduction in vacuolar membrane PE levels while total PE levels are not significantly affected. (1138 aa) | ||||
FMC1 | ATP synthase assembly factor FMC1, mitochondrial; Mitochondrial matrix protein; required for assembly or stability at high temperature of the F1 sector of mitochondrial F1F0 ATP synthase; null mutant temperature sensitive growth on glycerol is suppressed by multicopy expression of Odc1p. (155 aa) | ||||
ATP12 | Protein ATP12, mitochondrial; Assembly factor for F1 sector of mitochondrial F1F0 ATP synthase; conserved protein; required for assembly of alpha and beta subunits into F1 sector of mitochondrial F1F0 ATP synthase; human homolog ATPAF2 can complement yeast atp12 mutant; mutation of human homolog reduces active ATP synthase levels and is associated with the disorder ATPAF2 deficiency. (325 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) | ||||
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) | ||||
ATP7 | Subunit d of the stator stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis. (174 aa) | ||||
HSP104 | Disaggregase; heat shock protein that cooperates with Ydj1p (Hsp40) and Ssa1p (Hsp70) to refold and reactivate previously denatured, aggregated proteins; responsive to stresses including: heat, ethanol, and sodium arsenite; involved in [PSI+] propagation; protein becomes more abundant and forms cytoplasmic foci in response to DNA replication stress; potentiated Hsp104p variants decrease TDP-43 proteotoxicity by eliminating its cytoplasmic aggregation; Belongs to the ClpA/ClpB family. (908 aa) | ||||
ATP14 | Subunit h of the F0 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; protein abundance increases in response to DNA replication stress. (124 aa) | ||||
ATP10 | Mitochondrial ATPase complex subunit ATP10; Assembly factor for the F0 sector of mitochondrial F1F0 ATP synthase; mitochondrial inner membrane protein; interacts genetically with ATP6; Belongs to the ATP10 family. (279 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) | ||||
ATP25 | ATPase synthesis protein 25, mitochondrial; Mitochondrial protein required for the stability of Oli1p (Atp9p) mRNA; also required for the Oli1p ring formation; YMR098C is not an essential gene. (612 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) | ||||
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) | ||||
POR1 | Mitochondrial porin (voltage-dependent anion channel); outer membrane protein required for maintenance of mitochondrial osmotic stability and mitochondrial membrane permeability; couples the glutathione pools of the intermembrane space (IMS) and the cytosol; interacts with Om45 and Om14 in the outer membrane; phosphorylated; protein abundance increases in response to DNA replication stress. (283 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) | ||||
ATP11 | Protein ATP11, mitochondrial; Molecular chaperone; required for the assembly of alpha and beta subunits into the F1 sector of mitochondrial F1F0 ATP synthase; N-terminally propionylated in vivo. (318 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) | ||||
RPT4 | 26S proteasome subunit RPT4; ATPase of the 19S regulatory particle of the 26S proteasome; one of six ATPases of the regulatory particle; involved in degradation of ubiquitinated substrates; contributes preferentially to ERAD; required for spindle pole body duplication; mainly nuclear localization. (437 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) | ||||
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) | ||||
ATP15 | Epsilon 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 eukaryotic ATPase epsilon family. (62 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) | ||||
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) | ||||
ATP8 | ATP synthase protein 8; Subunit 8 of the F0 sector of mitochondrial F1F0 ATP synthase; encoded on the mitochondrial genome; ATP8 and ATP6 mRNAs are not translated in the absence of the F1 sector of ATPase. (48 aa) |