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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Mitochondrial ribosomal protein of the large subunit; also involved in homologous recombination in mitochondria; required for recombination-dependent mtDNA partitioning; involved in stimulation of mitochondrial DNA replication in response to oxidative stress.

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.

Mitochondrial DNA polymerase gamma; single subunit of mitochondrial DNA polymerase in yeast, in contrast to metazoan complex of catalytic and accessory subunits; polymorphic in yeast, petites occur more frequently in some lab strains; human ortholog POLG complements yeast mip1 mutant; mutations in human POLG associated with Alpers-Huttenlocher syndrome (AHS), progressive external ophthalmoplegia (PEO), parkinsonism, other mitochondrial diseases.

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.

Maintenance of mitochondrial morphology protein 1; ER integral membrane protein, ERMES complex subunit; ERMES 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; Belongs to the MMM1 family.

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.

DNA helicase, potent G-quadruplex DNA binder/unwinder; possesses strand annealing activity; promotes DNA synthesis during break-induced replication; important for crossover recombination; translation from different start sites produces mitochondrial and nuclear forms; nuclear form is a catalytic inhibitor of telomerase; mitochondrial form involved in DNA repair and recombination; mutations affect Zn, Fe homeostasis; regulated by Rad53p-dependent phosphorylation in rho0 cells.

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.

Mitochondrial RNA polymerase; single subunit enzyme similar to those of T3 and T7 bacteriophages; requires a specificity subunit encoded by MTF1 for promoter recognition; Mtf1p interacts with and stabilizes the Rpo41p-promoter complex, enhancing DNA bending and melting to facilitate pre-initiation open complex formation; Rpo41p also synthesizes RNA primers for mitochondrial DNA replication.

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.

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.

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.

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.

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.

Heat shock protein 60, mitochondrial; Tetradecameric mitochondrial chaperonin; required for ATP-dependent folding of precursor polypeptides and complex assembly; prevents aggregation and mediates protein refolding after heat shock; role in mtDNA transmission; phosphorylated.

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.

Heat shock protein 78, mitochondrial; Oligomeric mitochondrial matrix chaperone; cooperates with Ssc1p in mitochondrial thermotolerance after heat shock; able to prevent the aggregation of misfolded proteins as well as resolubilize protein aggregates.

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.

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.

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.

DnaJ homolog 1, mitochondrial; Co-chaperone that stimulates HSP70 protein Ssc1p ATPase activity; involved in protein folding/refolding in the mitochodrial matrix; required for proteolysis of misfolded proteins; member of the HSP40 (DnaJ) family of chaperones.

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.

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.