1.14.99.60: 3-demethoxyubiquinol 3-hydroxylase
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For detailed information about 3-demethoxyubiquinol 3-hydroxylase, go to the full flat file.
Word Map on EC 1.14.99.60
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1.14.99.60
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elegans
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lifespan
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nematode
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longevity
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ubiquinone
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worm
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long-lived
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demethoxyubiquinone
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dauer
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life-extending
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coq
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defecation
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gerontogenes
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analysis
- 1.14.99.60
- elegans
-
lifespan
- nematode
-
longevity
- ubiquinone
-
worm
-
long-lived
-
demethoxyubiquinone
-
dauer
-
life-extending
- coq
-
defecation
-
gerontogenes
- analysis
Reaction
Synonyms
2-nonaprenyl-3-methyl-6-methoxy-1,4-benzoquinol hydroxylase, 6-methoxy-3-methyl-2-(all-trans-polyprenyl)-1,4-benzoquinol 5-hydroxylase, Cat5, clk-1, CLK1, COQ7, ubiF
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1.14.99.60 3-demethoxyubiquinol 3-hydroxylaseAdvanced search results
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General Information on EC 1.14.99.60 - 3-demethoxyubiquinol 3-hydroxylase
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GENERAL INFORMATION 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
malfunction
the ubiF mutant shows poor growth on glucose and no growth on succinate, the ubiF mutant accumulates 2-octaprenyl-3-methyl-6-methoxy-1,4-benzoquinone
physiological function
physiological function
the enzyme catalyzes a reaction in the ubiquinone biosynthesis pathway
physiological function
a plasmid encoding CLK-1 supports aerobic respiration on a nonfermentable carbon source of the Escherichia coli ubiF mutant strain and rescues the ability to synthesize ubiquinone
physiological function
COQ7-deficient mouse embryos fail to survive beyond embryonic day 10.5, exhibit small-sized body and delayed embryogenesis. COQ7-deficient neuroepithelial cells fail to show the radial arrangement in the developing cerebral wall, aborting neurogenesis at E10.5. The enlarged mitochondria with vesicular cristae and enlarged lysosomes fill with disrupted membranes, which is consistent with mitochondriopathy. COQ7-deficient embryos fail to synthesize CoQ9, but instead yield demethoxyubiquinone 9
physiological function
deletion of the chromosomal Coq7 gene generates respiration defective yeast mutants deficient in ubiquinone. Coq mutants accumulate an early intermediate in the ubiquinone biosynthetic pathway, 3-hexaprenyl-4-hydroxybenzoate
physiological function
enzyme is directly involved in ubiquinone biosynthesis. The defect in gluconeogenic gene activation in Coq7/Cat5 null mutants is a general consequence of a defect in respiration
physiological function
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mutation lacking UbiF activity accumulate 2-octaprenyl-3-methyl-6-methoxy-1,4-benzoquinone
physiological function
promoter-driven COQ7/CLK-1 expression providing low levels of transgene expression rescues COQ7-deficient mice from embryonic lethality. The mice show a concomitant decrease in CoQ9, mitochondrial respiratory enzyme activity and the generation of reactive oxygen species in the mitochondria
physiological function
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the Escherichia coli UbiF gene expressed at low copy restores growth of Coq7 point mutant E194K on medium containing a non-fermentable carbon source, but fails to rescue a coq7 null mutant. Expression of UbiF from a multicopy vector restores growth and coenzyme Q synthesis for both mutants, although with a higher efficiency in the point mutant. Addition of coenzyme Q to the growth media also stabilizes the Coq3 and Coq4 polypeptides in the coq7 null mutant
physiological function
the gene complements an Escherichia coli mutant that lacks 5-demethoxyubiquinone hydroxylase UbiF
physiological function
the human CLK-1 gene is able to functionally complement Saccharomyces cerevisiae Coq7 deletion mutants
physiological function
transgenic expression of mouse CoQ7 in Caenorhabditis elegans completely rescues the slowed rhythmic behaviors of Clk-1 mutants such as defecation. In life-span analysis, transgenic expression reverts the extended life span of Clk-1 to a comparable level with wild-type control
physiological function
Caenorhabditis elegans Bristol 2
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a plasmid encoding CLK-1 supports aerobic respiration on a nonfermentable carbon source of the Escherichia coli ubiF mutant strain and rescues the ability to synthesize ubiquinone
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