5C53.31

ICD-11 MMS 2026-01

Mitochondrial protein import disorders

Description

This refers to disorders in the enzyme that belongs to the family of hydrolases, specifically those acting on acid anhydrides to catalyse transmembrane movement of substances.

Index terms

  • Mitochondrial protein import disorders

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

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

Foundation entity

Mitochondrial protein import disorders – http://id.who.int/icd/entity/112110122

Part of: 5C53.3: Disorders of mitochondrial membrane transport › 5C53: Inborn errors of energy metabolism › 05: Endocrine, nutritional or metabolic diseases