Ctns
entrez
83429
canonical
uniprot
P57757
canonical
ensembl
ENSMUSG00000005949
resolver
ensp
ENSMUSP00000006103
resolver
ensp
ENSMUSP00000104116
resolver
ensp
ENSMUSP00000247567
resolver
enst
ENSMUST00000006103
resolver
enst
ENSMUST00000108476
resolver
genesymbol
Ctns
resolver
uniprot
Q542U5
resolver
uniprot_entry
CTNS_MOUSE
resolver
uniprot_entry
Q542U5_MOUSE
resolver
activity_regulation
ACTIVITY REGULATION: Switches between a lumen- and a cytosol-open conformation: pH induces conformational changes and shifts the equilibrium to facilitate the transition between the lumen- and cytosol-open conformation, thereby promoting cystine transport. Protonation of specific aspartate residues (Asp-205, Asp-305 and Asp-346) favors the cytosol-open conformation. {ECO:0000250|UniProtKB:O60931}.
uniprot
proteins
amino_acid_sequence
MRRNWLLILTLFLLMFIEKYESTVSLTAPPTVKLENGSSTNVDITLGHPLNSTLVITFEVTFRSKNLTIVELPDEVIVPRGEKNASFQVTSQNIGQVTVFLHGNHSNQTCPRIRFLVIHSRIVSIINQVIGWIYFMAWSVSFYPQVIQNWRRKSVIGLSFDFLALNLTGFVAYSVFNIGLLWVPYIQEEFLLKYPNGVNPVDSNDAFFSLHAVALTLIVILQCCLYERGNQRVSWPSIGFLVLAWLFVLVTMIVAAVGITTWLQFLFCFSYIKLIITLIKYFPQAYMNFYYKSTKGWSIGGVLLDFTGGSFSLLQMFLQSYNNDQWTLIFGDPTKFGLGVFTIFFDVVFFIQHFYLYRKKPGYDQLN
uniprot
proteins
function
FUNCTION: Cystine/H(+) symporter that mediates export of cystine, the oxidized dimer of cysteine, from lysosomes (PubMed:12370309). Plays an important role in melanin synthesis by catalyzing cystine export from melanosomes, possibly by inhibiting pheomelanin synthesis (PubMed:22649030). In addition to cystine export, also acts as a positive regulator of mTORC1 signaling in kidney proximal tubular cells, via interactions with components of the v-ATPase and Ragulator complexes (PubMed:26449607). Also involved in small GTPase-regulated vesicle trafficking and lysosomal localization of LAMP2A, independently of cystine transporter activity (PubMed:28465352). {ECO:0000269|PubMed:12370309, ECO:0000269|PubMed:22649030, ECO:0000269|PubMed:26449607, ECO:0000269|PubMed:28465352}.
uniprot
proteins
mass_dalton
42203
uniprot
proteins
mutagenesis
MUTAGEN 280; /note="K->R: Abolished cystine transport. Abolished interaction with components of the v-ATPase and Ragulator complexes. Does not affect ability to lysosomal localization of LAMP2A."; /evidence="ECO:0000269|PubMed:26449607, ECO:0000269|PubMed:28465352
uniprot
proteins
protein_family
Cystinosin family
uniprot
proteins
pubmed
11121245
uniprot
proteins
pubmed
12370309
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
17977621
uniprot
proteins
pubmed
19349973
uniprot
proteins
pubmed
21897743
uniprot
proteins
pubmed
22649030
uniprot
proteins
pubmed
26449607
uniprot
proteins
pubmed
28465352
uniprot
proteins
sequence_length
367
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Lysosome membrane {ECO:0000269|PubMed:12370309}; Multi-pass membrane protein {ECO:0000255}. Melanosome membrane {ECO:0000250|UniProtKB:O60931}; Multi-pass membrane protein {ECO:0000255}. Note=AP-3 complex is required for localization to the lysosome. {ECO:0000250|UniProtKB:O60931}.
uniprot
proteins
transmembrane_region
TRANSMEM 126..150; /note="Helical"; /evidence="ECO:0000250|UniProtKB:O60931"; TRANSMEM 160..179; /note="Helical"; /evidence="ECO:0000250|UniProtKB:O60931"; TRANSMEM 203..225; /note="Helical"; /evidence="ECO:0000250|UniProtKB:O60931"; TRANSMEM 235..257; /note="Helical"; /evidence="ECO:0000250|UniProtKB:O60931"; TRANSMEM 264..289; /note="Helical"; /evidence="ECO:0000250|UniProtKB:O60931"; TRANSMEM 299..308; /note="Helical"; /evidence="ECO:0000250|UniProtKB:O60931"; TRANSMEM 332..354; /note="Helical"; /evidence="ECO:0000250|UniProtKB:O60931
uniprot
proteins