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5C53.22 Coenzyme Q10 deficiency

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

This is a deficiency in a 1,4-benzoquinone, where Q refers to the quinone chemical group, and 10 refers to the number of isoprenyl chemical subunits in its tail. This oil-soluble, vitamin-like substance is present in most eukaryotic cells, primarily in the mitochondria. It is a component of the electron transport chain and participates in aerobic cellular respiration, generating energy in the form of ATP.

code elsewhere

• Cerebellar atrophy - ataxia - seizures  (LD90.Y)

synonyms

• Coenzyme Q10 deficiency
• CoQ10 deficiency
• Early-onset ataxia with oculomotor apraxia and hypoalbuminaemia
• Ataxia - oculomotor apraxia type 1
• Deafness - encephaloneuropathy - obesity - valvulopathy

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