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MS
SLC3A1 / SLC7A9 AR unreviewed

Cystinuria

Renal amino acid transport

Enzyme or protein defect

Dibasic amino acid transporter (COLA)

Accumulates or becomes deficient

Cystine in urine

Key features

Recurrent hexagonal cystine kidney stones

Management

Hydration, urinary alkalinisation, penicillamine/tiopronin

Not clinical guidance. Written from standard clinical biochemistry, not from the uploaded documents, and marked unreviewed. Teaching-level reference for study and database seeding only. It must not be used to manage a patient.

Recent literature

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