Article

The CoQ biosynthetic di-iron carboxylate hydroxylase COQ7 is inhibited by in vivo metalation with manganese but remains function by metalation with cobalt

2022-07-13

Abstract excerpt

<h4>ABSTRACT</h4> COQ7 is a mitochondrial hydroxylase that catalyzes the penultimate step of the biosynthesis of coenzyme Q (CoQ; ubiquinone). CoQ is an obligate component of the mitochondrial electron transport chain and an antioxidant. CoQ deficiencies due to mutations in CoQ biosynthetic enzymes are severe genetic disorders often manifesting as mitochondrial disease syndrome. COQ7 is part of the relatively rar...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: ce1501bd-7184-5e0c-a993-5c1b7a1a9642
DOI: 10.1101/2022.07.12.499729

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

The CoQ biosynthetic di-iron carboxylate hydroxylase COQ7 is inhibited by in vivo metalation with manganese but remains function by metalation with cobaltDOI 10.1101/2022.07.12.499729Read

A new member of the family of di-iron carboxylate proteins. Coq7 (clk-1), a membrane-bound hydroxylase involved in ubiquinone biosynthesis.Semantic proximity Read

The phosphatase Ptc7 induces coenzyme Q biosynthesis by activating the hydroxylase Coq7 in yeast.Semantic proximity Read

The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene.Semantic proximity Read

COQ4 is required for the oxidative decarboxylation of the C1 carbon of Coenzyme Q in eukaryotic cellsSemantic proximity Read

Overexpression of the Coq8 kinase in Saccharomyces cerevisiae coq null mutants allows for accumulation of diagnostic intermediates of the coenzyme Q6 biosynthetic pathway.Semantic proximity Read

Use of a tyrosine hydroxylase mutant enzyme with reduced metal affinity allows detection of activity with cobalt in place of iron.Semantic proximity Read

Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1.Semantic proximity Read

New insights into the mechanism of nickel insertion into carbon monoxide dehydrogenase: analysis of Rhodospirillum rubrum carbon monoxide dehydrogenase variants with substituted ligands to the [Fe3S4] portion of the active-site C-cluster.Semantic proximity Read

Regulation of coenzyme Q biosynthesis in yeast: a new complex in the block.Semantic proximity Read

Coenzyme Q biosynthesis: Coq6 is required for the C5-hydroxylation reaction and substrate analogs rescue Coq6 deficiency.Semantic proximity Read

Uptake of exogenous coenzyme Q and transport to mitochondria is required for bc1 complex stability in yeast coq mutants.Semantic proximity Read

Coenzyme Q supplementation or over-expression of the yeast Coq8 putative kinase stabilizes multi-subunit Coq polypeptide complexes in yeast coq null mutants.Semantic proximity Read