Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

5-demethoxyubiquinone hydroxylase, mitochondrial; Protein required for ubiquinone (Coenzyme Q) biosynthesis; localizes to the matrix face of the mitochondrial inner membrane in a large complex with ubiquinone biosynthetic enzymes; required for gluconeogenic gene activation; Belongs to the COQ7 family.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Hexaprenyl pyrophosphate synthetase; catalyzes the first step in ubiquinone (coenzyme Q) biosynthesis; Belongs to the FPP/GGPP synthase family.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

4-hydroxybenzoate polyprenyltransferase, mitochondrial; Para hydroxybenzoate polyprenyl transferase; catalyzes the second step in ubiquinone (coenzyme Q) biosynthesis; human COQ2, mutations in which are implicated in an increased risk of mutiple-system atrophy, can complement a yeast coq2 null mutant.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

O-methyltransferase; catalyzes two different O-methylation steps in ubiquinone (Coenzyme Q) biosynthesis; component of a mitochondrial ubiquinone-synthesizing complex; phosphoprotein; Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Protein with a role in ubiquinone (Coenzyme Q) biosynthesis; possibly functioning in stabilization of Coq7p; located on matrix face of mitochondrial inner membrane; component of a mitochondrial ubiquinone-synthesizing complex; human homolog COQ4 can complement yeast coq4 null mutant.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial; 2-hexaprenyl-6-methoxy-1,4-benzoquinone methyltransferase; involved in ubiquinone (Coenzyme Q) biosynthesis; localizes to the matrix face of the mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; respiratory defect of the null mutant is partially complemented by human COQ5; Belongs to the class I-like SAM-binding methyltransferase superfamily. MenG/UbiE family.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Flavin-dependent monooxygenase involved in ubiquinone biosynthesis; responsible for hydroxylation at position C5 and deamination at C4 during ubiquinone (Coenzyme Q) biosynthesis; localizes to matrix face of mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; human homolog COQ6 can complement yeast null mutant and is implicated in steroid-resistant nephrotic syndrome (SRNS).

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Atypical kinase COQ8, mitochondrial; ATPase required for ubiquinone biosynthesis and respiratory growth; maintains levels of CoQ biosynthetic proteins; binds to CoQ biosynthesis intermediates; UbiB protein kinase-like family member that lacks canonical protein kinase activity; similar to prokaryotic proteins involved in ubiquinone biosynthesis; human homolog ADCK3 complements a coq8 null, is associated with CoQ and respiratory-chain deficiencies, and is mutated in autosomal-recessive cerebellar ataxia type 2.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Protein required for ubiquinone biosynthesis and respiratory growth; localizes to matrix face of mitochondrial inner membrane in a large complex with ubiquinone biosynthetic enzymes; ubiquinone is also known as coenzyme Q; human homolog COQ9 can complement yeast coq9 null mutant.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Cytochrome 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.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Cytochrome c isoform 2, expressed under hypoxic conditions; also known as iso-2-cytochrome c; electron carrier of the mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; protein abundance increases in response to DNA replication stress; CYC7 has a paralog, CYC1, that arose from the whole genome duplication.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Cytochrome c1, heme protein, mitochondrial; Cytochrome c1; component of the mitochondrial respiratory chain; expression is regulated by the heme-activated, glucose-repressed Hap2p/3p/4p/5p CCAAT-binding complex.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Ubiquinol-cytochrome-c reductase; a Rieske iron-sulfur protein of the mitochondrial cytochrome bc1 complex; transfers electrons from ubiquinol to cytochrome c1 during respiration; during import, Rip1p is first imported into the mitochondrial matrix where it is processed, acquires its Fe-S cluster, and is folded, then is translocated into the inner membrane by the action of a homo-oligomer of Bcs1p, and finally is delivered by Bcs1p to Complex III for assembly.

Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family.

Adrenodoxin homolog, mitochondrial; Ferredoxin of the mitochondrial matrix; required for formation of cellular iron-sulfur proteins; involved in heme A biosynthesis; human homolog FDX1L can complement yeast by allowing growth during down-regulation of yeast YAH1; Belongs to the adrenodoxin/putidaredoxin family.

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.

5-demethoxyubiquinone hydroxylase, mitochondrial; Protein required for ubiquinone (Coenzyme Q) biosynthesis; localizes to the matrix face of the mitochondrial inner membrane in a large complex with ubiquinone biosynthetic enzymes; required for gluconeogenic gene activation; Belongs to the COQ7 family.

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.

Hexaprenyl pyrophosphate synthetase; catalyzes the first step in ubiquinone (coenzyme Q) biosynthesis; Belongs to the FPP/GGPP synthase family.

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.

4-hydroxybenzoate polyprenyltransferase, mitochondrial; Para hydroxybenzoate polyprenyl transferase; catalyzes the second step in ubiquinone (coenzyme Q) biosynthesis; human COQ2, mutations in which are implicated in an increased risk of mutiple-system atrophy, can complement a yeast coq2 null mutant.

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.

O-methyltransferase; catalyzes two different O-methylation steps in ubiquinone (Coenzyme Q) biosynthesis; component of a mitochondrial ubiquinone-synthesizing complex; phosphoprotein; Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family.

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.

Protein with a role in ubiquinone (Coenzyme Q) biosynthesis; possibly functioning in stabilization of Coq7p; located on matrix face of mitochondrial inner membrane; component of a mitochondrial ubiquinone-synthesizing complex; human homolog COQ4 can complement yeast coq4 null mutant.

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.

2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial; 2-hexaprenyl-6-methoxy-1,4-benzoquinone methyltransferase; involved in ubiquinone (Coenzyme Q) biosynthesis; localizes to the matrix face of the mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; respiratory defect of the null mutant is partially complemented by human COQ5; Belongs to the class I-like SAM-binding methyltransferase superfamily. MenG/UbiE family.