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.

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.

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.

Endonuclease III homolog 1; DNA N-glycosylase and apurinic/apyrimidinic (AP) lyase; involved in base excision repair; acts in both nucleus and mitochondrion; creates a double-strand break at mtDNA origins that stimulates replication in response to oxidative stress; required for maintaining mitochondrial genome integrity; NTG1 has a paralog, NTG2, that arose from the whole genome duplication; Belongs to the Nth/MutY 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.

Putative uncharacterized protein Q0092, mitochondrial; Dubious open reading frame; unlikely to encode a functional protein, based on available experimental and comparative sequence data.

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.

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.

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.

Subunit 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.

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.

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.

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.

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.

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.

Mitochondrial ribosomal protein of the small subunit; mitochondrially-encoded; polymorphic in different strains due to variation in number of AAT (asparagine) codons; translated near the mitochondrial inner membrane; may have a role in loss of mitochondrial DNA under stress conditions.

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.

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.

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.

Subunit 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.

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.

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.

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.

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.

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.

Putative uncharacterized protein Q0092, mitochondrial; Dubious open reading frame; unlikely to encode a functional protein, based on available experimental and comparative sequence data.

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.

Mitochondrial ribosomal protein of the small subunit; mitochondrially-encoded; polymorphic in different strains due to variation in number of AAT (asparagine) codons; translated near the mitochondrial inner membrane; may have a role in loss of mitochondrial DNA under stress conditions.

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.

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.

DNA damage-inducible protein DIN7; Mitochondrial nuclease functioning in DNA repair and replication; modulates the stability of the mitochondrial genome, induced by exposure to mutagens, also induced during meiosis at a time nearly coincident with commitment to recombination; DIN7 has a paralog, EXO1, that arose from the whole genome duplication; Belongs to the XPG/RAD2 endonuclease family.

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.

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.

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.

Endonuclease III homolog 1; DNA N-glycosylase and apurinic/apyrimidinic (AP) lyase; involved in base excision repair; acts in both nucleus and mitochondrion; creates a double-strand break at mtDNA origins that stimulates replication in response to oxidative stress; required for maintaining mitochondrial genome integrity; NTG1 has a paralog, NTG2, that arose from the whole genome duplication; Belongs to the Nth/MutY family.