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

Stable Identifier
R-HSA-8932851
Type
Reaction [transition]
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

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

Proc. Natl. Acad. Sci. U.S.A. 2012
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
basic amino acid transmembrane transporter activity of PQLC2 [lysosomal membrane]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
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Reviewed
Created