PQLC2 transports L-Arg,L-His,L-Lys from lysosomal lumen to cytosol

Stable Identifier
R-HSA-8932851
Type
Reaction
Species
Homo sapiens
Compartment
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Lysosomal amino acid transporter 1 homolog (PQLC2) is a lysosomal membrane-associated protein that mediates the efflux of the cationic amino acids L-Arg, L-His and L-Lys from the lysosomal lumen to the cytosol, contributing to their homeostasis in cells. PQLC2 belongs to a family of heptahelical membrane proteins, together with the founding member cystinosin, the lysosomal cystine exporter defective in cystinosis. The family are characterised by a duplicated motif termed the PQ loop (Jezegou et al. 2012).

Literature References
PubMed ID Title Journal Year
23169667 Heptahelical protein PQLC2 is a lysosomal cationic amino acid exporter underlying the action of cysteamine in cystinosis therapy

Kieffer-Jaquinod, S, Llinares, E, Andre, B, Chadefaux-Vekemans, B, Anne, C, Sagné, C, Aupetit, J, Jézégou, A, Journet, A, Debacker, C, O'Regan, S, Gasnier, B, Chabli, A

Proc. Natl. Acad. Sci. U.S.A. 2012
Participants
Participates
Catalyst Activity

basic amino acid transmembrane transporter activity of PQLC2 [lysosomal membrane]

Orthologous Events
Cross References
Rhea
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