Coenzyme Q10 deficiency

Coenzyme Q₁₀ deficiency
Coenzyme Q10 deficiency
Other names	Leigh syndrome with nephrotic syndrome
	Ubiquinone

Coenzyme Q₁₀ deficiency is a deficiency of coenzyme Q₁₀.

It can be associated with mutations in COQ2, APTX, PDSS2, PDSS1, CABC1, and COQ9.^[1] Some forms may be more treatable than other mitochondrial diseases.^[2]

Classification	D OMIM: 607426 614650 616276 MeSH: C564403
External resources	Orphanet: 255249

v t e Disorders of citric acid cycle and electron transport chain
Citric acid cycle	Pyruvate dehydrogenase deficiency Fumarase deficiency
Electron transport chain	Coenzyme Q10 deficiency Björnstad syndrome GRACILE syndrome Leigh disease

v t e Mitochondrial diseases
Carbohydrate metabolism	PCD PDHA
Primarily nervous system	Leigh disease LHON NARP
Myopathies	KSS Mitochondrial encephalomyopathy MELAS MERRF CPEO
No primary system	DAD MNGIE Pearson syndrome
Chromosomal	OPA1 Kjer's optic neuropathy SARS2 HUPRA syndrome TIMM8A Mohr–Tranebjærg syndrome
see also mitochondrial proteins

External links