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SLC66 Lysosomal amino acid transporters C

Unless otherwise stated all data on this page refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).


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This is a family of 5 evolutionarily related proteins. Their structural similarities suggest that they are transporters. Biochemical evidence supports transporter activity for SLC66A1 (LAAT1) and SLC66A4 (CTNS; Cystinosin), primarily exporting amino acids from the lysosome to the cytoplasm. The functions of the 3 remaining members of the family are undetermined.


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solute carrier family 66 member 1 / SLC66A1 C Show summary »

Target Id 3160
Nomenclature solute carrier family 66 member 1
Systematic nomenclature SLC66A1
Previous and unofficial names LAAT-1 | LAAT1 | lysosomal amino acid transporter 1 homolog | PQLC2 | PQ loop repeat containing 2
Genes SLC66A1 (Hs), Slc66a1 (Mm), Slc66a1 (Rn)
Ensembl ID ENSG00000040487 (Hs), ENSMUSG00000028744 (Mm), ENSRNOG00000017706 (Rn)
UniProtKB AC Q6ZP29 (Hs), Q8C4N4 (Mm), B0BMY1 (Rn)
Comment Responsible for lysine and arginine export from lysosomes [7]. Functions as a pH-sensitive uniporter [6]. Transports cysteamine-cysteine mixed disulfide, structurally similar to lysine, which is a chemical intermediate formed during cysteamine therapy of cystinosis [3]. Acts to recruit the C9orf72-SMCR8-WDR41 complex to the cytoplasmic side of the lysosome upon cationic amino acid starvation, as the initiator of the signaling cascade [1,10].

solute carrier family 66 member 2 / SLC66A2 C Show summary »

solute carrier family 66 member 3 / SLC66A3 C Show summary »

cystinosin, lysosomal cystine transporter / SLC66A4 C Show summary »

mannose-P-dolichol utilization defect 1 / SLC66A5 C Show summary »

Further reading

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NC-IUPHAR subcommittee and family contributors

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How to cite this family page

Database page citation (select format):

Concise Guide to PHARMACOLOGY citation:

Alexander SPH, Fabbro D, Kelly E, Mathie AA, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Davies JA et al. (2023) The Concise Guide to PHARMACOLOGY 2023/24: Transporters. Br J Pharmacol. 180 Suppl 2:S374-469.