5C53.22

ICD-11 MMS 2026-01

Coenzyme Q10 deficiency

Description

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.

Index terms

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

Exclusions from above levels

  • http://id.who.int/icd/entity/1191191920 – Disorders of lipoprotein metabolism or certain specified lipidaemias
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
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  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5C53.20 – Mitochondrial DNA depletion syndromes
  • 5C53.21 – Multiple mitochondrial DNA deletion syndromes
  • 5C53.23 – Mitochondrial protein translation defects
  • 5C53.24 – Leigh syndrome
  • 5C53.25 – Isolated ATP synthase deficiency
  • 5C53.2Y – Other specified disorders of mitochondrial oxidative phosphorylation
  • 5C53.2Z – Disorders of mitochondrial oxidative phosphorylation, unspecified

Foundation entity

Coenzyme Q10 deficiency – http://id.who.int/icd/entity/1251664337

Part of: 5C53.2: Disorders of mitochondrial oxidative phosphorylation › 5C53: Inborn errors of energy metabolism › 05: Endocrine, nutritional or metabolic diseases