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8dkm
From Proteopedia
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| - | '''Unreleased structure''' | ||
| - | + | ==Cryo-EM structure of cystine-bound cystinosin in a lumen-open state== | |
| - | + | <StructureSection load='8dkm' size='340' side='right'caption='[[8dkm]], [[Resolution|resolution]] 3.39Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[8dkm]] is a 3 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Mus_musculus Mus musculus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=8DKM OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8DKM FirstGlance]. <br> | |
| - | + | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=IYY:L-cystine'>IYY</scene></td></tr> | |
| - | [[Category: | + | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8dkm FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8dkm OCA], [https://pdbe.org/8dkm PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8dkm RCSB], [https://www.ebi.ac.uk/pdbsum/8dkm PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8dkm ProSAT]</span></td></tr> |
| - | [[Category: | + | </table> |
| - | [[Category: Li | + | == Disease == |
| + | [[https://www.uniprot.org/uniprot/CTNS_HUMAN CTNS_HUMAN]] Juvenile nephropathic cystinosis;Ocular cystinosis;Infantile nephropathic cystinosis. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. | ||
| + | == Function == | ||
| + | [[https://www.uniprot.org/uniprot/CTNS_HUMAN CTNS_HUMAN]] Cystine/H(+) symporter that mediates export of cystine, the oxidized dimer of cysteine, from lysosomes (PubMed:11689434, PubMed:18337546, PubMed:22232659, PubMed:29467429, PubMed:33208952, PubMed:15128704). 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 (By similarity). Also involved in small GTPase-regulated vesicle trafficking and lysosomal localization of LAMP2A, independently of cystine transporter activity (By similarity).[UniProtKB:P57757]<ref>PMID:11689434</ref> <ref>PMID:15128704</ref> <ref>PMID:18337546</ref> <ref>PMID:22232659</ref> <ref>PMID:22649030</ref> <ref>PMID:29467429</ref> <ref>PMID:33208952</ref> | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Homo sapiens]] | ||
| + | [[Category: Large Structures]] | ||
| + | [[Category: Mus musculus]] | ||
| + | [[Category: Li X]] | ||
| + | [[Category: Schmiege P]] | ||
Current revision
Cryo-EM structure of cystine-bound cystinosin in a lumen-open state
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