protein 888 words KG: CTNSPROTEIN 2026-03-21
kind:proteinsection:proteinsstate:published
Contents

Cystinosin (CTNS Protein)

🔬 Protein Info
Gene SymbolCTNSPROTEIN
Protein TypeTransporter
Functionis a lysosomal membrane transporter protein critical for cystine efflux from lysosomes.
Subcellular Localizationto the lysosomal membrane, where it functions as an H⁺-driven cystine transporte
Molecular Weight42 kDa
GeneCardsCTNSPROTEIN
Human Protein AtlasCTNSPROTEIN
N-terminal domainLocated in the lysosomal lumen, contains 7 N-glycosylation sites
Seven transmembrane helices (TM1-TM7)Span the lysosomal membrane to form the cystine translocation pathway
Two PQ-loop motifsConserved Pro-Gln sequences in TM2 and TM5 essential for transport
C-terminal tailCytoplasmic, contains the GYDQL lysosomal sorting motif
Intralysosomal loopsLarge glycosylated loops between TMs protect against lysosomal proteases
Autophagic fluxReduced autophagosome-lysosome fusion
Chaperone-mediated autophagyImpaired LAMP2A-dependent CMA
Endosomal traffickingDelayed endosomal maturation and cargo sorting
Associated Diseasesneurodegeneration
DatabasesGeneCardsUniProtNCBI GeneHPASTRING

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