Cystinosis [cystinóza]

topical
16
Terms

nefropatická cystinóza

 

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

Persistent link   https://www.medvik.cz/link/D003554
Definition

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.

DUI
D003554 MeSH Browser
CUI
M0005556

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