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NDI1 NDI1 COX1 COX1 COX2 COX2 MDM10 MDM10 COR1 COR1 PTH2 PTH2 ATP3 ATP3 PGS1 PGS1 ATP16 ATP16 COX9 COX9 NDE2 NDE2 TPI1 TPI1 SDH4 SDH4 FMN1 FMN1 ATP17 ATP17 QCR7 QCR7 COX4 COX4 COX13 COX13 MPC3 MPC3 COX6 COX6 TOM71 TOM71 MPC2 MPC2 TIM44 TIM44 TIM17 TIM17 CYC1 CYC1 CYT2 CYT2 OAC1 OAC1 SDH3 SDH3 COX12 COX12 XDJ1 XDJ1 ICT1 ICT1 UPS2 UPS2 UPS1 UPS1 COX8 COX8 NDE1 NDE1 TOM40 TOM40 AEP2 AEP2 COX5A COX5A TOM70 TOM70 MDM12 MDM12 SDH5 SDH5 ATP19 ATP19 TOM6 TOM6 MDM32 MDM32 MCP1 MCP1 COT1 COT1 ATP15 ATP15 ATP20 ATP20 YME1 YME1 QCR2 QCR2
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NDI1Rotenone-insensitive NADH-ubiquinone oxidoreductase, mitochondrial; NADH:ubiquinone oxidoreductase; transfers electrons from NADH to ubiquinone in respiratory chain but does not pump protons, in contrast to higher eukaryotic multisubunit respiratory complex I; upon apoptotic stress, is activated in mitochondria by N-terminal cleavage, then translocates to cytoplasm to induce apoptosis; homolog of human AIFM2; yeast NDI1 complements several phenotypes of human cell line with mutated MT-ND4, implicated in Leber hereditary optic neuropathy. (513 aa)
COX1Subunit 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)
COX2Subunit 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)
MDM10Mitochondrial 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)
COR1Core subunit of the ubiquinol-cytochrome c reductase complex; the ubiquinol-cytochrome c reductase complex (bc1 complex) is a component of the mitochondrial inner membrane electron transport chain. (457 aa)
PTH2Peptidyl-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)
ATP3Gamma 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)
PGS1CDP-diacylglycerol--glycerol-3-phosphate 3-phosphatidyltransferase; Phosphatidylglycerolphosphate synthase; catalyzes the synthesis of phosphatidylglycerolphosphate from CDP-diacylglycerol and sn-glycerol 3-phosphate in the first committed and rate-limiting step of cardiolipin biosynthesis. (521 aa)
ATP16Delta 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)
COX9Subunit VIIa of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain. (59 aa)
NDE2External NADH-ubiquinone oxidoreductase 2, mitochondrial; Mitochondrial external NADH dehydrogenase; catalyzes the oxidation of cytosolic NADH; Nde1p and Nde2p are involved in providing the cytosolic NADH to the mitochondrial respiratory chain; NDE2 has a paralog, NDE1, that arose from the whole genome duplication. (545 aa)
TPI1Triose 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)
SDH4Succinate 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)
FMN1Riboflavin kinase, produces riboflavin monophosphate (FMN); FMN is a necessary cofactor for many enzymes; predominantly localizes to the microsomal fraction and also found in the mitochondrial inner membrane; human RFK functionally complements the lethality of the null mutation. (218 aa)
ATP17Subunit f of the F0 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis. (101 aa)
QCR7Subunit 7 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; oriented facing the mitochondrial matrix; N-terminus appears to play a role in complex assembly; Belongs to the UQCRB/QCR7 family. (127 aa)
COX4Subunit 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)
COX13Subunit VIa of cytochrome c oxidase; present in a subclass of cytochrome c oxidase complexes that may have a role in mimimizing generation of reactive oxygen species; not essential for cytochrome c oxidase activity but may modulate activity in response to ATP; required for assembly of Rcf2p into cytochrome c oxidase - cytochrome bc1 supercomplexes. (129 aa)
MPC3Highly 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)
COX6Subunit 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)
TOM71Protein TOM71; Mitochondrial outer membrane protein; probable minor component of the TOM (translocase of outer membrane) complex responsible for recognition and import of mitochondrially directed proteins; TOM71 has a paralog, TOM70, that arose from the whole genome duplication. (639 aa)
MPC2Highly 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)
TIM44Mitochondrial import inner membrane translocase subunit TIM44; Essential component of the TIM23 complex; tethers the import motor and regulatory factors (PAM complex) to the translocation channel (Tim23p-Tim17p core complex); TIM23 complex is short for the translocase of the inner mitochondrial membrane; Belongs to the Tim44 family. (431 aa)
TIM17Mitochondrial 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)
CYC1Cytochrome 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)
CYT2Cytochrome 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)
OAC1Mitochondrial inner membrane transporter; transports oxaloacetate, sulfate, thiosulfate, and isopropylmalate; member of the mitochondrial carrier family. (324 aa)
SDH3Subunit 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)
COX12Subunit VIb of cytochrome c oxidase; cytochrome c oxidase is also known as respiratory Complex IV and is the terminal member of the mitochondrial inner membrane electron transport chain; required for assembly of cytochrome c oxidase but not required for activity after assembly; phosphorylated; easily released from the intermembrane space, suggesting a loose association with Complex IV. (83 aa)
XDJ1DnaJ protein homolog XDJ1; Chaperone with a role in facilitating mitochondrial protein import; ascomycete-specific member of the DnaJ-like family, closely related to Ydj1p; predicted to be C-terminally prenylated; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies. (459 aa)
ICT11-acylglycerol-3-phosphate O-acyltransferase ICT1; Lysophosphatidic acid acyltransferase; responsible for enhanced phospholipid synthesis during organic solvent stress; null displays increased sensitivity to Calcofluor white; highly expressed during organic solvent stress; ICT1 has a paralog, ECM18, that arose from the whole genome duplication; human ABHD5 can complement ict1 null mutant; Belongs to the peptidase S33 family. ABHD4/ABHD5 subfamily. (394 aa)
UPS2Mitochondrial intermembrane space protein; involved in phospholipid metabolism; forms complex with Mdm35p that transfers phosphatidylserine from outer membrane to inner membrane for phosphatidylethanolamine synthesis; null mutant has defects in mitochondrial morphology; similar to Ups1p, Ups3p and to human PRELI; UPS2 has a paralog, UPS3, that arose from the whole genome duplication; Belongs to the slowmo family. (230 aa)
UPS1Protein UPS1, mitochondrial; Phosphatidic acid transfer protein; plays a role in phospholipid metabolism by transporting phosphatidic acid from the outer to the inner mitochondrial membrane; localizes to the mitochondrial intermembrane space; null mutant has altered cardiolipin and phosphatidic acid levels; ortholog of human PRELI; Belongs to the slowmo family. (175 aa)
COX8Subunit VIII of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain. (78 aa)
NDE1Mitochondrial external NADH dehydrogenase; type II NAD(P)H:quinone oxidoreductase that catalyzes the oxidation of cytosolic NADH; Nde1p and Nde2p provide cytosolic NADH to the mitochondrial respiratory chain; NDE1 has a paralog, NDE2, that arose from the whole genome duplication. (560 aa)
TOM40Mitochondrial 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)
AEP2ATPase expression protein 2, mitochondrial; Mitochondrial protein; likely involved in translation of the mitochondrial OLI1 mRNA; exhibits genetic interaction with the OLI1 mRNA 5'-untranslated leader; Belongs to the AEP2 family. (580 aa)
COX5ASubunit Va of cytochrome c oxidase; cytochrome c oxidase is the terminal member of the mitochondrial inner membrane electron transport chain; Cox5Ap is predominantly expressed during aerobic growth while its isoform Vb (Cox5Bp) is expressed during anaerobic growth; COX5A has a paralog, COX5B, that arose from the whole genome duplication. (153 aa)
TOM70Mitochondrial 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)
MDM12Mitochondrial distribution and morphology protein 12; Mitochondrial outer membrane protein, ERMES complex subunit; required for transmission of mitochondria to daughter cells; required for mitophagy; may influence import and assembly of outer membrane beta-barrel proteins; ERMES complex is often co-localized with peroxisomes and with concentrated areas of pyruvate dehydrogenase; Belongs to the MDM12 family. (271 aa)
SDH5Succinate dehydrogenase assembly factor 2, mitochondrial; Protein required for flavinylation of Sdh1p; binds to Sdh1p and promotes FAD cofactor attachment, which is necessary for succinate dehydrogenase (SDH) complex assembly and activity; mutations in human ortholog PGL2 are associated with neuroendocrine tumors (paraganglioma). (162 aa)
ATP19Subunit k of the mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; associated only with the dimeric form of ATP synthase; Belongs to the ATP19 family. (68 aa)
TOM6Mitochondrial 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)
MDM32Mitochondrial distribution and morphology protein 32; Mitochondrial inner membrane protein with similarity to Mdm31p; required for normal mitochondrial morphology and inheritance; interacts genetically with MMM1, MDM10, MDM12, and MDM34; variation between SK1 and S288C at residues 182 and 262 impacts invasive growth and mitochondrial network structure; Belongs to the MDM31/MDM32 family. (622 aa)
MCP1MDM10-complementing protein 1; Mitochondrial protein of unknown function involved in lipid homeostasis; integral membrane protein that localizes to the mitochondrial outer membrane; involved in mitochondrial morphology; interacts genetically with MDM10, and other members of the ERMES complex; contains five predicted transmembrane domains. (302 aa)
COT1Cobalt uptake protein COT1; Vacuolar transporter that mediates zinc transport into the vacuole; overexpression confers resistance to cobalt and rhodium; protein abundance increases in response to DNA replication stress; COT1 has a paralog, ZRC1, that arose from the whole genome duplication; Belongs to the cation diffusion facilitator (CDF) transporter (TC 2.A.4) family. SLC30A subfamily. (439 aa)
ATP15Epsilon 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)
ATP20Subunit 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)
YME1Catalytic 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)
QCR2Subunit 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)
Your Current Organism:
Saccharomyces cerevisiae
NCBI taxonomy Id: 4932
Other names: ATCC 18824, Candida robusta, Mycoderma cerevisiae, NRRL Y-12632, S. cerevisiae, Saccharomyces capensis, Saccharomyces italicus, Saccharomyces oviformis, Saccharomyces uvarum var. melibiosus, yeast
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