Cystinosin, the protein defective in cystinosis, is a H+-driven lysosomal cystine transporter

EMBO Journal

Volumn 20, Issue 21, 2001, Pages 5940-5949

  Kalatzis, Vasiliki ^a   Cherqui, Stéphanie ^a   Antignac, Corinne ^a   Gasnier, Bruno ^b  

^a INSERM   (France)

^b INST DE BIOLOGIE PHYSICO CHIMIQUE   (France)

Author keywords

Cystine; Cystinosis; H+ symporter; Lysosome; Transporter

Indexed keywords

CELL PROTEIN; CYSTINE; CYSTINOSIN; UNCLASSIFIED DRUG;

AMINO ACID TRANSPORT; ANIMAL CELL; ARTICLE; CARBOXY TERMINAL SEQUENCE; CYSTINOSIS; LYSOSOME; LYSOSOME STORAGE DISEASE; NONHUMAN; PH; POINT MUTATION; PRIORITY JOURNAL; PROTEIN EXPRESSION; SACCHAROMYCES CEREVISIAE;

AMINO ACID MOTIFS; ANIMALS; BIOLOGICAL TRANSPORT, ACTIVE; CARRIER PROTEINS; CELL MEMBRANE; COS CELLS; CYSTINE; CYSTINOSIS; GLYCOPROTEINS; HYDROGEN-ION CONCENTRATION; LYSOSOMES; MEMBRANE PROTEINS; MEMBRANE TRANSPORT PROTEINS; MUTAGENESIS, SITE-DIRECTED; POINT MUTATION; PROTONS; RECOMBINANT PROTEINS; SEQUENCE DELETION; SUBSTRATE SPECIFICITY; TRANSFECTION;

ANIMALIA; MAMMALIA; SACCHAROMYCES CEREVISIAE;

EID: 0035503565     PISSN: 02614189     EISSN: None     Source Type: Journal    
DOI: 10.1093/emboj/20.21.5940     Document Type: Article

References (49)

1. Requirement of the Lec35 gene for all known classes of monosaccharide-P-dolichol-dependent glycosyltransferase reactions in mammals
2. CTNS mutations in patients with cystinosis
3. Severity of phenotype in cystinosis varies with mutations in the CTNS gene: Predicted effect on the model of cystinosin
4. Identification of an amino acid transporter associated with the cystinuria-related type II membrane glycoprotein
5. The targeting of cystinosin to the lysosomal membrane requires a tyrosine-based signal and a novel sorting motif
6. +AT) of rBAT. International Cystinuria Consortium
7. The cytoskeleton of digitonin-treated rat hepatocytes
8. Molecular characterization of CTNS deletions in nephropathic cystinosis: Development of a PCR-based detection assay
9. pH effects on cystine transport in lysosome-rich leucocyte granular fractions
10. Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis
11. Defective cystine exodus from isolated lysosome-rich fractions of cystinotic leucocytes
12. Characteristics of cystine counter-transport in normal and cystinotic lysosome-rich leucocyte granular fractions
13. Lysosomal transport disorders: Cystinosis and sialic acid storage disorders
14. Cystine exodus from normal leucocytes is stimulated by MgATP
15. Characterization of the lysosomal cystine transport system in mouse L-929 fibroblasts
16. ERS1, a seven transmembrane domain protein from Saccharomyces cerevisiae
17. Purification of the lysosomal sialic acid transporter. Functional characteristics of a monocarboxylate transporter
18. Cystine transport in purified rat liver lysosomes
19. Cystine accumulation and loss in normal, heterozygous and cystinotic fibroblasts
20. ATP-dependent lysosomal cystine efflux is defective in cystinosis
21. Proton-translocating ATPase and lysosomal cystine transport
22. Lysosomal transport disorders
23. Vacuolar and plasma membrane proton-adenosinetriphosphatases
24. Molecular biology of mammalian plasma membrane amino acid transporters
25. The molecular bases of cystinuria and lysinuric protein intolerance
26. Sorting of soluble ER proteins in yeast
27. Luminal heterodimeric amino acid transporter defective in cystinuria
28. Amino acid transport by lysosomes
29. Ionophorous antibiotics as models for biological transport
30. Structure and function of plasma membrane amino acid, oligopeptide and sucrose transporters from higher plants
31. Transmembrane movement of a water-soluble analogue of mannosylphosphoryldolichol is mediated by an endoplasmic reticulum protein
32. A family of yeast proteins mediating bidirectional vacuolar amino acid transport
33. Identification and characterization of a lysosomal transporter for small neutral amino acids
34. Cloning and expression of a plasma membrane cystine/glutamate exchange transporter composed of two distinct proteins
35. xCt cystine transporter expression in HEK293 cells: Pharmacology and localization
36. CTNS mutations in an American-based population of cystinosis patients
37. Lysosomal cystine transport. Effect of intralysosomal pH and membrane potential
38. Thin-layer chromatographic separation of cystine and the N-ethylmaleimide adducts of cysteine and glutathíonen
39. Cystine accumulation and clearance by normal and cystinotic leukocytes exposed to cystine dimethyl ester
40. Mutations of CTNS causing intermediate cystinosis
41. 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
42. A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis
43. Characteristics of taurine transport in rat liver lysosomes
44. A genomic view of yeast membrane transporters
45. A new gene, encoding an anion transporter, is mutated in sialic acid storage diseases
46. Expression cloning of a novel suppressor of the Lec15 and Lec35 glycosylation mutations of Chinese hamster ovary cells
47. A block at Man5GlcNAc2-pyrophosphoryldolichol in intact but not disrupted castanospermine and swainsonine-resistant Chinese hamster ovary cells
48. Homologues of archaeal rhodopsins in plants, animals and fungi: Structural and functional predications for a putative fungal chaperone protein
49. Rapid separation of particulate components and soluble cytoplasm of isolated rat-liver cells