Details for: CTNS

Gene ID: 1497

Symbol: CTNS

Ensembl ID: ENSG00000040531

Description: cystinosin, lysosomal cystine transporter

Associated with

Other Information

Genular Protein ID: 3658933797

Symbol: CTNS_HUMAN

Name: N/A

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 9537412

Title: A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis.

PubMed ID: 9537412

DOI: 10.1038/ng0498-319

PubMed ID: 10673275

Title: The genomic region encompassing the nephropathic cystinosis gene (CTNS): complete sequencing of a 200-kb segment and discovery of a novel gene within the common cystinosis-causing deletion.

PubMed ID: 10673275

DOI: 10.1101/gr.10.2.165

PubMed ID: 14702039

Title: Complete sequencing and characterization of 21,243 full-length human cDNAs.

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 16625196

Title: DNA sequence of human chromosome 17 and analysis of rearrangement in the human lineage.

PubMed ID: 16625196

DOI: 10.1038/nature04689

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 10068513

Title: Identification and detection of the common 65-kb deletion breakpoint in the nephropathic cystinosis gene (CTNS).

PubMed ID: 10068513

DOI: 10.1006/mgme.1998.2790

PubMed ID: 28082515

Title: Impact of cystinosin glycosylation on protein stability by differential dynamic stable isotope labeling by amino acids in cell culture (SILAC).

PubMed ID: 28082515

DOI: 10.1074/mcp.m116.063867

PubMed ID: 10571941

Title: CTNS mutations in patients with cystinosis.

PubMed ID: 10571941

DOI: 10.1002/(sici)1098-1004(199912)14:6<454::aid-humu2>3.0.co;2-h

PubMed ID: 11689434

Title: Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter.

PubMed ID: 11689434

DOI: 10.1093/emboj/20.21.5940

PubMed ID: 11150305

Title: The targeting of cystinosin to the lysosomal membrane requires a tyrosine-based signal and a novel sorting motif.

PubMed ID: 11150305

DOI: 10.1074/jbc.m010562200

PubMed ID: 17897319

Title: Integral and associated lysosomal membrane proteins.

PubMed ID: 17897319

DOI: 10.1111/j.1600-0854.2007.00643.x

PubMed ID: 18337546

Title: Identification and subcellular localization of a new cystinosin isoform.

PubMed ID: 18337546

DOI: 10.1152/ajprenal.00413.2007

PubMed ID: 22649030

Title: Cystinosin is a melanosomal protein that regulates melanin synthesis.

PubMed ID: 22649030

DOI: 10.1096/fj.11-201376

PubMed ID: 22544350

Title: Distribution of cystinosin-LKG in human tissues.

PubMed ID: 22544350

DOI: 10.1007/s00418-012-0958-8

PubMed ID: 22232659

Title: Mechanism of proton/substrate coupling in the heptahelical lysosomal transporter cystinosin.

PubMed ID: 22232659

DOI: 10.1073/pnas.1115581109

PubMed ID: 25753619

Title: Lysosomal targeting of cystinosin requires AP-3.

PubMed ID: 25753619

DOI: 10.1111/tra.12277

PubMed ID: 27148969

Title: Carboxyl-terminal SSLKG motif of the human cystinosin-LKG plays an important role in plasma membrane sorting.

PubMed ID: 27148969

DOI: 10.1371/journal.pone.0154805

PubMed ID: 29467429

Title: A genetic screen for investigating the human lysosomal cystine transporter, cystinosin.

PubMed ID: 29467429

DOI: 10.1038/s41598-018-21483-x

PubMed ID: 33208952

Title: MFSD12 mediates the import of cysteine into melanosomes and lysosomes.

PubMed ID: 33208952

DOI: 10.1038/s41586-020-2937-x

PubMed ID: 36113465

Title: Structure and mechanism of human cystine exporter cystinosin.

PubMed ID: 36113465

DOI: 10.1016/j.cell.2022.08.020

PubMed ID: 9792862

Title: CTNS mutations in an American-based population of cystinosis patients.

PubMed ID: 9792862

DOI: 10.1086/302118

PubMed ID: 10482956

Title: Molecular analysis of cystinosis: probable Irish origin of the most common French Canadian mutation.

PubMed ID: 10482956

DOI: 10.1038/sj.ejhg.5200349

PubMed ID: 10444339

Title: Mutations of CTNS causing intermediate cystinosis.

PubMed ID: 10444339

DOI: 10.1006/mgme.1999.2876

PubMed ID: 10556299

Title: Severity of phenotype in cystinosis varies with mutations in the CTNS gene: predicted effect on the model of cystinosin.

PubMed ID: 10556299

DOI: 10.1093/hmg/8.13.2507

PubMed ID: 10625078

Title: Ocular nonnephropathic cystinosis: clinical, biochemical, and molecular correlations.

PubMed ID: 10625078

DOI: 10.1203/00006450-200001000-00007

PubMed ID: 12204010

Title: Analysis of the CTNS gene in patients of German and Swiss origin with nephropathic cystinosis.

PubMed ID: 12204010

DOI: 10.1002/humu.9063

PubMed ID: 12442267

Title: Identification of 14 novel CTNS mutations and characterization of seven splice site mutations associated with cystinosis.

PubMed ID: 12442267

DOI: 10.1002/humu.10141

PubMed ID: 12825071

Title: Mutational spectrum of the CTNS gene in Italy.

PubMed ID: 12825071

DOI: 10.1038/sj.ejhg.5200993

PubMed ID: 15128704

Title: Molecular pathogenesis of cystinosis: effect of CTNS mutations on the transport activity and subcellular localization of cystinosin.

PubMed ID: 15128704

DOI: 10.1093/hmg/ddh152

PubMed ID: 19852576

Title: Characterization of CTNS mutations in Arab patients with cystinosis.

PubMed ID: 19852576

DOI: 10.3109/13816810903200953

PubMed ID: 22450360

Title: Two novel CTNS mutations in cystinosis patients in Thailand.

PubMed ID: 22450360

DOI: 10.1016/j.gene.2012.03.047

PubMed ID: 21786142

Title: Genetic basis of cystinosis in Turkish patients: a single-center experience.

PubMed ID: 21786142

DOI: 10.1007/s00467-011-1942-6

Sequence Information:

  • Length: 367
  • Mass: 41738
  • Checksum: 9343889CD7576908
  • Sequence:
  • MIRNWLTIFI LFPLKLVEKC ESSVSLTVPP VVKLENGSST NVSLTLRPPL NATLVITFEI 
    TFRSKNITIL ELPDEVVVPP GVTNSSFQVT SQNVGQLTVY LHGNHSNQTG PRIRFLVIRS 
    SAISIINQVI GWIYFVAWSI SFYPQVIMNW RRKSVIGLSF DFVALNLTGF VAYSVFNIGL 
    LWVPYIKEQF LLKYPNGVNP VNSNDVFFSL HAVVLTLIII VQCCLYERGG QRVSWPAIGF 
    LVLAWLFAFV TMIVAAVGVT TWLQFLFCFS YIKLAVTLVK YFPQAYMNFY YKSTEGWSIG 
    NVLLDFTGGS FSLLQMFLQS YNNDQWTLIF GDPTKFGLGV FSIVFDVVFF IQHFCLYRKR 
    PGYDQLN

Genular Protein ID: 848862966

Symbol: A0A0S2Z3I9_HUMAN

Name: N/A

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 26871637

Title: Widespread Expansion of Protein Interaction Capabilities by Alternative Splicing.

PubMed ID: 26871637

DOI: 10.1016/j.cell.2016.01.029

Sequence Information:

  • Length: 400
  • Mass: 45039
  • Checksum: 47AF39E14090DFC2
  • Sequence:
  • MIRNWLTIFI LFPLKLVEKC ESSVSLTVPP VVKLENGSST NVSLTLRPPL NATLVITFEI 
    TFRSKNITIL ELPDEVVVPP GVTNSSFQVT SQNVGQLTVY LHGNHSNQTG PRIRFLVIRS 
    SAISIINQVI GWIYFVAWSI SFYPQVIMNW RRKSVIGLSF DFVALNLTGF VAYSVFNIGL 
    LWVPYIKEQF LLKYPNGVNP VNSNDVFFSL HAVVLTLIII VQCCLYERGG QRVSWPAIGF 
    LVLAWLFAFV TMIVAAVGVT TWLQFLFCFS YIKLAVTLVK YFPQAYMNFY YKSTEGWSIG 
    NVLLDFTGGS FSLLQMFLQS YNNDQWTLIF GDPTKFGLGV FSIVFDVVFF IQHFCLYRKR 
    PGLQAARTGS GSRLRQDWAP SLQPKALPQT TSVSASSLKG

Genular Protein ID: 1457886422

Symbol: I3L4A9_HUMAN

Name: N/A

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 16625196

Title: DNA sequence of human chromosome 17 and analysis of rearrangement in the human lineage.

PubMed ID: 16625196

DOI: 10.1038/nature04689

Sequence Information:

  • Length: 253
  • Mass: 28630
  • Checksum: 79F1BB80FFF3D836
  • Sequence:
  • MNWRRKSVIG LSFDFVALNL TGFVAYSVFN IGLLWVPYIK EQFLLKYPNG VNPVNSNDVF 
    FSLHAVVLTL IIIVQCCLYE RGGQRVSWPA IGFLVLAWLF AFVTMIVAAV GVTTWLQFLF 
    CFSYIKLAVT LVKYFPQAYM NFYYKSTEGW SIGNVLLDFT GGSFSLLQMF LQSYNNDQWT 
    LIFGDPTKFG LGVFSIVFDV VFFIQHFCLY RKRPGLQAAR TGSGSRLRQD WAPSLQPKAL 
    PQTTSVSASS LKG

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.