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GGC1 | Mitochondrial GTP/GDP carrier protein 1; Mitochondrial GTP/GDP transporter; essential for mitochondrial genome maintenance; has a role in mitochondrial iron transport; member of the mitochondrial carrier family. (300 aa) | ||||
KGD2 | 2-oxoglutarate dehydrogenase E2 component (dihydrolipoamide succinyltransferase); Dihydrolipoyl transsuccinylase; component of the mitochondrial alpha-ketoglutarate dehydrogenase complex, which catalyzes the oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA in the TCA cycle; phosphorylated. (463 aa) | ||||
NUM1 | Protein required for nuclear migration; component of the mitochondria-ER-cortex-ancor (MECA); required for the association of mitochondria with the cell cortex and for accurate distribution of mitochondrial network; interacts with Mdm36p to link the ER and mitochondria at the cortex; localizes to the mother cell cortex and the bud tip; may mediate interactions of dynein and cytoplasmic microtubules with the cell cortex. (2748 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) | ||||
LPD1 | Dihydrolipoyl dehydrogenase, mitochondrial; Dihydrolipoamide dehydrogenase; the lipoamide dehydrogenase component (E3) of the pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase multi-enzyme complexes; PDH complex is concentrated in spots within the mitochondrial matrix, often near the ERMES complex and near peroxisomes; LPD1 has a paralog, IRC15, that arose from the whole genome duplication; Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family. (499 aa) | ||||
ACT1 | Actin; structural protein involved in cell polarization, endocytosis, and other cytoskeletal functions. (375 aa) | ||||
MNP1 | Mitochondrial ribosomal protein of the large subunit; has similarity to E. coli L7/L12 and human MRPL7 ribosomal proteins; associates with the mitochondrial nucleoid; required for normal respiratory growth. (194 aa) | ||||
YPT32 | GTP-binding protein YPT32/YPT11; Rab family GTPase involved in the exocytic pathway; mediates intra-Golgi traffic or the budding of post-Golgi vesicles from the trans-Golgi; protein abundance increases in response to DNA replication stress; YPT32 has a paralog, YPT31, that arose from the whole genome duplication. (222 aa) | ||||
MDM34 | Mitochondrial distribution and morphology protein 34; Mitochondrial component of the ERMES complex; links the ER to mitochondria and may promote inter-organellar calcium and phospholipid exchange as well as coordinating mitochondrial DNA replication and growth; required for mitophagy; ERMES complex is often co-localized with peroxisomes and with concentrated areas of pyruvate dehydrogenase. (459 aa) | ||||
HMG1 | HMG-CoA reductase; catalyzes conversion of HMG-CoA to mevalonate, which is a rate-limiting step in sterol biosynthesis; one of two isozymes; localizes to nuclear envelope; overproduction induces formation of karmellae; forms foci at nuclear periphery upon DNA replication stress; HMG1 has a paralog, HMG2, that arose from the whole genome duplication; human homolog HMGCR can complement yeast hmg1 mutant. (1054 aa) | ||||
HMG2 | HMG-CoA reductase; converts HMG-CoA to mevalonate, a rate-limiting step in sterol biosynthesis; one of two isozymes; overproduction induces assembly of peripheral ER membrane arrays and short nuclear-associated membrane stacks; forms foci at nuclear periphery upon DNA replication stress; HMG2 has a paralog, HMG1, that arose from the whole genome duplication; human homolog HMGCR can complement yeast hmg2 mutant. (1045 aa) | ||||
ILV5 | Ketol-acid reductoisomerase, mitochondrial; Acetohydroxyacid reductoisomerase and mtDNA binding protein; involved in branched-chain amino acid biosynthesis and maintenance of wild-type mitochondrial DNA; found in mitochondrial nucleoids. (395 aa) | ||||
ACO1 | Aconitate hydratase, mitochondrial; Aconitase; required for the tricarboxylic acid (TCA) cycle and also independently required for mitochondrial genome maintenance; component of the mitochondrial nucleoid; mutation leads to glutamate auxotrophy; human homolog ACO2 can complement yeast null mutant. (778 aa) | ||||
MMR1 | Mitochondrial MYO2 receptor-related protein 1; Phosphorylated protein of the mitochondrial outer membrane; localizes only to mitochondria of the bud; interacts with Myo2p to mediate mitochondrial distribution to buds; mRNA is targeted to the bud via the transport system involving She2p. (491 aa) | ||||
DNM1 | Dynamin-related protein DNM1; Dynamin-related GTPase involved in mitochondrial organization; required for mitochondrial fission and inheritance; self assembles on the cytoplasmic face of mitochondrial tubules at sites where division will occur; participates in endocytosis and regulates peroxisome fission along with Vps1p; mutants in the human ortholog DNM1L, which mediates mitochondrial fission, peroxisomal division, autophagy, and mitophagy, are associated with slowly progressive infantile encephalopathy. (757 aa) | ||||
CAF4 | CCR4-associated factor 4; WD40 repeat-containing protein associated with the CCR4-NOT complex; interacts in a Ccr4p-dependent manner with Ssn2p; also interacts with Fis1p, Mdv1p and Dnm1p and plays a role in mitochondrial fission; CAF4 has a paralog, MDV1, that arose from the whole genome duplication. (643 aa) | ||||
CCE1 | Mitochondrial cruciform cutting endonuclease; cleaves Holliday junctions formed during recombination of mitochondrial DNA; CCE1 has a paralog, MRS1, that arose from the whole genome duplication. (353 aa) | ||||
MGM101 | Mitochondrial genome maintenance protein MGM101; Protein with a role in mitochondrial DNA recombinational repair; also involved in interstrand cross-link repair; binds to and catalyzes the annealing of single-stranded mtDNA; oligomerizes to form rings and filaments; related to Rad52-type recombination proteins, with limited overall similarity but sharing conserved functionally important residues; component of the mitochondrial nucleoid, required for the repair of oxidative mtDNA damage and mitochondrial genome maintenance; Belongs to the MGM101 family. (269 aa) | ||||
MPH1 | 3'-5' DNA helicase involved in error-free bypass of DNA lesions; binds flap DNA, stimulates activity of Rad27p and Dna2p; prevents crossovers between ectopic sequences by removing substrates for Mus81-Mms4 or Rad1-Rad10 cleavage; homolog of human FANCM Fanconi anemia protein that is involved in stabilizing and remodeling blocked replication forks; member of SF2 DExD/H superfamily of helicases; nonsense or missense mutations in FANCM can make people more likely to get cancer. (993 aa) | ||||
KGD1 | 2-oxoglutarate dehydrogenase, mitochondrial; Subunit of the mitochondrial alpha-ketoglutarate dehydrogenase complex; catalyzes a key step in the tricarboxylic acid (TCA) cycle, the oxidative decarboxylation of alpha-ketoglutarate to form succinyl-CoA. (1014 aa) | ||||
FIS1 | Protein involved in mitochondrial fission and peroxisome abundance; may have a distinct role in tethering protein aggregates to mitochondria in order to retain them in the mother cell; required for localization of Dnm1p and Mdv1p during mitochondrial division; mediates ethanol-induced apoptosis and ethanol-induced mitochondrial fragmentation; Belongs to the FIS1 family. (155 aa) | ||||
Q0092 | Putative uncharacterized protein Q0092, mitochondrial; Dubious open reading frame; unlikely to encode a functional protein, based on available experimental and comparative sequence data. (46 aa) | ||||
MDM10 | Mitochondrial 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) | ||||
FZO1 | Mitofusin; integral membrane protein involved in mitochondrial outer membrane tethering and fusion; role in mitochondrial genome maintenance; efficient tethering and degradation of Fzo1p requires an intact N-terminal GTPase domain; targeted for destruction by the ubiquitin ligase SCF-Mdm30p and the cytosolic ubiquitin-proteasome system; Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family. Mitofusin subfamily. (855 aa) | ||||
CIT2 | Citrate synthase, peroxisomal isozyme involved in glyoxylate cycle; catalyzes condensation of acetyl coenzyme A and oxaloacetate to form citrate; expression is controlled by Rtg1p and Rtg2p transcription factors; SCF-Ucc1 regulates level of Cit2p to maintain citrate homeostasis; oxaloacetate-dependent positive feedback loop inhibits Cit2p ubiquitination; CIT2 has a paralog, CIT1, that arose from the whole genome duplication. (460 aa) | ||||
NHP6A | Non-histone chromosomal protein 6A; High-mobility group (HMG) protein; binds to and remodels nucleosomes; involved in recruiting FACT and other chromatin remodelling complexes to chromosomes; functionally redundant with Nhp6Bp; required for transcriptional initiation fidelity of some tRNA genes; homologous to mammalian HMGB1 and HMGB2; NHP6A has a paralog, NHP6B, that arose from the whole genome duplication; protein abundance increases in response to DNA replication stress. (93 aa) | ||||
CIT3 | Dual specificity mitochondrial citrate and methylcitrate synthase; catalyzes the condensation of acetyl-CoA and oxaloacetate to form citrate and that of propionyl-CoA and oxaloacetate to form 2-methylcitrate. (486 aa) | ||||
ALD4 | Mitochondrial aldehyde dehydrogenase; required for growth on ethanol and conversion of acetaldehyde to acetate; phosphorylated; activity is K+ dependent; utilizes NADP+ or NAD+ equally as coenzymes; expression is glucose repressed; can substitute for cytosolic NADP-dependent aldehyde dehydrogenase when directed to the cytosol; human homolog ALDH2 can complement yeast ald4 mutant. (519 aa) | ||||
MYO2 | Myosin-2; Type V myosin motor involved in actin-based transport of cargos; required for the polarized delivery of secretory vesicles, the vacuole, late Golgi elements, peroxisomes, and the mitotic spindle; MYO2 has a paralog, MYO4, that arose from the whole genome duplication; Belongs to the TRAFAC class myosin-kinesin ATPase superfamily. Myosin family. (1574 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) | ||||
MDM12 | Mitochondrial 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) | ||||
CIT1 | Citrate synthase, mitochondrial; Citrate synthase; catalyzes the condensation of acetyl coenzyme A and oxaloacetate to form citrate; the rate-limiting enzyme of the TCA cycle; nuclear encoded mitochondrial protein; CIT1 has a paralog, CIT2, that arose from the whole genome duplication. (479 aa) | ||||
YPT11 | GTP-binding protein YPT11; Rab GTPase; Myo2p-binding protein implicated in mother-to-bud transport of cortical endoplasmic reticulum (ER), late Golgi, and mitochondria during cell division; function is regulated at multiple levels; abundance of active Ypt11p forms is controlled by phosphorylation status and degradation; normally a low-abundance protein whose ER localization is only detected when protein is highly over expressed. (417 aa) | ||||
YHM2 | Citrate and oxoglutarate carrier protein; exports citrate from and imports oxoglutarate into the mitochondrion, causing net export of NADPH reducing equivalents; also associates with mt nucleoids and has a role in replication and segregation of the mt genome; Belongs to the mitochondrial carrier (TC 2.A.29) family. (314 aa) | ||||
ALD3 | Cytoplasmic aldehyde dehydrogenase; involved in beta-alanine synthesis; uses NAD+ as the preferred coenzyme; very similar to Ald2p; expression is induced by stress and repressed by glucose. (506 aa) | ||||
PSO2 | DNA cross-link repair protein PSO2/SNM1; Nuclease required for DNA single- and double-strand break repair; acts at a post-incision step in repair of breaks that result from interstrand cross-links produced by a variety of mono- and bi-functional psoralen derivatives; induced by UV-irradiation; forms nuclear foci upon DNA replication stress; Belongs to the DNA repair metallo-beta-lactamase (DRMBL) family. (661 aa) | ||||
ABF2 | ARS-binding factor 2, mitochondrial; Mitochondrial DNA-binding protein; involved in mitochondrial DNA replication and recombination, member of HMG1 DNA-binding protein family; activity may be regulated by protein kinase A phosphorylation; ABF2 has a paralog, IXR1, that arose from the whole genome duplication; human homolog TFAM can complement yeast abf2 mutant, rescuing the loss-of-mitochondrial DNA phenotype in a yeast abf2 strain. (183 aa) | ||||
RAD52 | DNA repair and recombination protein RAD52; Protein that stimulates strand exchange; stimulates strand exchange by facilitating Rad51p binding to single-stranded DNA; anneals complementary single-stranded DNA; involved in the repair of double-strand breaks in DNA during vegetative growth and meiosis and UV induced sister chromatid recombination; Belongs to the RAD52 family. (471 aa) |