iDrugBank

Cystinosis

MESHD003554

A metabolic disease characterized by the defective transport of CYSTINE across the lysosomal membrane due to mutation of a membrane protein cystinosin. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. In the KIDNEY, nephropathic cystinosis is a common cause of RENAL FANCONI SYNDROME.

Synonyms

  • Cystine Storage Disease
  • Cystine Storage Diseases
  • Storage Disease, Cystine
  • Storage Diseases, Cystine
  • Lysosomal Cystine Transport Protein, Defect Of
  • Cystine Disease
  • Cystine Diseases
  • Cystinoses
  • Cystine Diathesis
  • Cystine Diatheses
  • Diatheses, Cystine
  • Diathesis, Cystine
  • Cystinosin, Defect of
  • Defect of Cystinosin
  • Nephropathic Cystinosis
  • Cystinosis, Nephropathic
  • Cystinoses, Nephropathic
  • Nephropathic Cystinoses

Source last updated 2026-05-24T15:27:57.172Z