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.

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.

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.

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.

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.

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).

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.

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.

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.

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.

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.

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.

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.

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).

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.

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.

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.

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.

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.

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.

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.

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).

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.

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.

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).

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.

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).

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.

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).

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.

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).

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.

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.

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.

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.

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.

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.

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.

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.

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).