5C53.3

ICD-11 MMS 2026-01

Disorders of mitochondrial membrane transport

Description

An inborn error of metabolism in proteins in the membranes of mitochondria, which serve to transport molecules and other factors such as ions into or out of the organelles

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

Child codes

  • 5C53.30 – Mitochondrial substrate carrier disorders
  • 5C53.31 – Mitochondrial protein import disorders
  • 5C53.3Y – Other specified disorders of mitochondrial membrane transport
  • 5C53.3Z – Disorders of mitochondrial membrane transport, unspecified

Sibling codes

  • 5C53.0 – Disorders of pyruvate metabolism
  • 5C53.1 – Disorders of the citric acid cycle
  • 5C53.2 – Disorders of mitochondrial oxidative phosphorylation
  • 5C53.4 – Disorders of creatine metabolism
  • 5C53.Y – Other specified inborn errors of energy metabolism
  • 5C53.Z – Inborn errors of energy metabolism, unspecified

Foundation entity

Disorders of mitochondrial membrane transport – http://id.who.int/icd/entity/1340308055

Part of: 5C53: Inborn errors of energy metabolism › 05: Endocrine, nutritional or metabolic diseases