2.A.43.2.1
The lysosomal lysine-arginine transporter, LAAT1, SLC66A1, PQ-loop repeat containing protein 2 (PQLC2) (291 aas; 7 TMSs). Also transports L-histidine, L-ornithine, the mixed disulfide of cysteine-cysteamine, a lysine analogue, as well as canavanine, a toxic arginine analogue. Cysteamine is used in the treatment of cystinosis (Liu et al., 2012; Jézégou et al. 2012).
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| Accession Number: | Q6ZP29 |
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| Protein Name: | PQ-loop repeat-containing protein 2 |
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| Length: | 291 |
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| Molecular Weight: | 31947.00 |
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| Species: | Homo sapiens (Human) [9606] |
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| Number of TMSs: | 7 |
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| Location1 / Topology2 / Orientation3: |
Membrane1 / Multi-pass membrane protein2 |
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| Substrate |
cysteamine, lysine, L-ornithine, L-canavanine, cysteine, arginine |
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1: MVWKKLGSRN FSSCPSGSIQ WIWDVLGECA QDGWDEASVG LGLISILCFA ASTFPQFIKA
61: YKTGNMDQAL SLWFLLGWIG GDSCNLIGSF LADQLPLQTY TAVYYVLADL VMLTLYFYYK
121: FRTRPSLLSA PINSVLLFLM GMACATPLLS AAGPVAAPRE AFRGRALLSV ESGSKPFTRQ
181: EVIGFVIGSI SSVLYLLSRL PQIRTNFLRK STQGISYSLF ALVMLGNTLY GLSVLLKNPE
241: EGQSEGSYLL HHLPWLVGSL GVLLLDTIIS IQFLVYRRST AASELEPLLP S