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Target intelligence / Profile preview
Demethoxyubiquinone Hydroxylase (CLK-1)
- Target
- CLK-1
- Molecular classification
- Enzyme, Oxidoreductase, Monooxygenase
01
Overview
Demethoxyubiquinone hydroxylase, or CLK-1, is a mitochondrial enzyme essential for ubiquinone (coenzyme Q10) biosynthesis. It catalyzes the hydroxylation of DMQH2, a key step in converting demethoxyubiquinone to ubiquinone. Ubiquinone is vital for electron transport and ATP production. Mutations or deficiencies in COQ7/CLK-1 are associated with rare metabolic disorders. CLK-1 also influences nuclear signaling pathways and lifespan determination. It is a potential therapeutic target via precursor supplementation or genetic correction mechanisms.
- Other names
- COQ75-demethoxyubiquinone hydroxylaseCAT5
02
Mechanism of action
N/A
03
Biological functions
Coenzyme Q10 biosynthesisMitochondrial electron transport chainATP production
04
Disease associations
Primary Coenzyme Q10 deficiency syndromesEncephalopathy-hypertrophic cardiomyopathy-renal disease spectrum
05
Safety considerations
Rare metabolic disorders associated with deficiencySupplementation strategies may not be universally effective
06
Biomarkers
Demethoxyubiquinone levelsUbiquinone levels
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