Heptahelical protein PQLC2 is a lysosomal cationic amino acid exporter underlying the action of cysteamine in cystinosis therapy
about
Cystinosis: a reviewRole of amino acid transporters in amino acid sensingLysosomal membrane proteins and their central role in physiologyRegulation of mitochondrial pyruvate uptake by alternative pyruvate carrier complexes.Endo-lysosomal dysfunction in human proximal tubular epithelial cells deficient for lysosomal cystine transporter cystinosinIntegrated electroosmotic perfusion of tissue with online microfluidic analysis to track the metabolism of cystamine, pantethine, and coenzyme A.Regulation of pyruvate metabolism and human diseaseRare inherited kidney diseases: challenges, opportunities, and perspectives.Structures of bacterial homologues of SWEET transporters in two distinct conformations.Treatment of corneal cystine crystal accumulation in patients with cystinosis.Lysosomal physiology.Clinical utility of chitotriosidase enzyme activity in nephropathic cystinosis.A mouse model suggests two mechanisms for thyroid alterations in infantile cystinosis: decreased thyroglobulin synthesis due to endoplasmic reticulum stress/unfolded protein response and impaired lysosomal processing.Hematopoietic Stem Cells Transplantation Can Normalize Thyroid Function in a Cystinosis Mouse ModelAn extended proteome map of the lysosomal membrane reveals novel potential transporters.Cystinosin is a Component of the Vacuolar H+-ATPase-Ragulator-Rag Complex Controlling Mammalian Target of Rapamycin Complex 1 Signaling.Functional role of oligomerization for bacterial and plant SWEET sugar transporter family.Cysteamine modulates oxidative stress and blocks myofibroblast activity in CKD.Cfs1p, a Novel Membrane Protein in the PQ-Loop Family, Is Involved in Phospholipid Flippase Functions in YeastSuccessful management of a neglected case of nephropathic cystinosis.Improving the prognosis of nephropathic cystinosis.Function and Regulation of Fungal Amino Acid Transporters: Insights from Predicted Structure.The renal Fanconi syndrome in cystinosis: pathogenic insights and therapeutic perspectives.A cysteamine-selective two-photon fluorescent probe for ratiometric bioimaging.Decoding the substrate supply to human neuronal nitric oxide synthase.Exploring genetic suppression interactions on a global scale.Clinical Practice: A Proposed Standardized Ophthalmological Assessment for Patients with Cystinosis.Cysteamine revisited: repair of arginine to cysteine mutations.Exacerbating and reversing lysosomal storage diseases: from yeast to humans.Lysosomal Targeting of Cystinosin Requires AP-3.Characterization of Avt1p as a vacuolar proton/amino acid antiporter in Saccharomyces cerevisiae.Thiol trapping and metabolic redistribution of sulfur metabolites enable cells to overcome cysteine overload.Rapid and efficient genome-wide characterization of Xanthomonas TAL effector genes.SNAT7 is the primary lysosomal glutamine exporter required for extracellular protein-dependent growth of cancer cells.The AP-3 adaptor complex mediates sorting of yeast and mammalian PQ-loop-family basic amino acid transporters to the vacuolar/lysosomal membrane.Effects of long-term cysteamine treatment in patients with cystinosis.A Genetic Screen for Investigating the Human Lysosomal CystineTransporter, Cystinosin.PQLC2 transports L-Arg;L-His;L-Lys from lysosomal lumen to cytosolNatural Variation in the Multidrug Efflux Pump SGE1 Underlies Ionic Liquid Tolerance in Yeast
P2860
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P248
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P2860
Heptahelical protein PQLC2 is a lysosomal cationic amino acid exporter underlying the action of cysteamine in cystinosis therapy
description
2012 nî lūn-bûn
@nan
2012 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2012 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
name
Heptahelical protein PQLC2 is ...... steamine in cystinosis therapy
@ast
Heptahelical protein PQLC2 is ...... steamine in cystinosis therapy
@en
Heptahelical protein PQLC2 is ...... teamine in cystinosis therapy.
@nl
type
label
Heptahelical protein PQLC2 is ...... steamine in cystinosis therapy
@ast
Heptahelical protein PQLC2 is ...... steamine in cystinosis therapy
@en
Heptahelical protein PQLC2 is ...... teamine in cystinosis therapy.
@nl
prefLabel
Heptahelical protein PQLC2 is ...... steamine in cystinosis therapy
@ast
Heptahelical protein PQLC2 is ...... steamine in cystinosis therapy
@en
Heptahelical protein PQLC2 is ...... teamine in cystinosis therapy.
@nl
P2093
P2860
P3181
P356
P1476
Heptahelical protein PQLC2 is ...... steamine in cystinosis therapy
@en
P2093
Adrien Jézégou
Agnès Journet
Allel Chabli
Bernadette Chadefaux-Vekemans
Bruno André
Bruno Gasnier
Christine Anne
Corinne Sagné
Cécile Debacker
Elisa Llinares
P2860
P304
P3181
P356
10.1073/PNAS.1211198109
P407
P577
2012-12-11T00:00:00Z