Diseases

Cystinosis

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

MeSH Record

Classification

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MeSH Record

Synonyms

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

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Hierarchy

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AMA Style

References

  1. National Library of Medicine. Cystinosis. Medical Subject Headings (MeSH). 2026. Unique ID D003554. http://id.nlm.nih.gov/mesh/2026/D003554
  2. Cystinosis. In: Wikipedia. https://en.wikipedia.org/wiki/Cystinosis
  3. Cystinosis. In: Wikidata. https://www.wikidata.org/wiki/Q1149042