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Lysosomal Cystine
- Molecular classification
- Amino Acid, Metabolite
01
Overview
Lysosomal cystine refers to the amino acid cystine that accumulates within lysosomes as a result of protein degradation. Under normal conditions, it is exported to the cytoplasm by cystinosin. Its roles include amino acid recycling, redox homeostasis, and metabolic adaptation during fasting. Its accumulation due to cystinosin deficiency causes cystinosis.
02
Mechanism of action
Cysteamine depletes intralysosomal free-cystine via thiol-disulfide exchange reactions with accumulated free-cystine.
03
Biological functions
Amino acid storageRedox homeostasisMetabolic adaptation during fastingPrecursor for glutathione synthesis
04
Disease associations
CystinosisKidney dysfunctionOxidative stress-related diseases
05
Safety considerations
Accumulation leads to cell death, particularly in kidney cellsDepletion of cellular glutathione (GSH)
06
Interacting drugs
Cysteamine
07
Biomarkers
Intralysosomal cystine levels
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