| scholarly article | Q13442814 |
| P50 | author | Stephanie Cherqui | Q46181476 |
| Sergio D Catz | Q55427359 | ||
| Gennaro Napolitano | Q62659350 | ||
| Celine J Rocca | Q64505813 | ||
| P2093 | author name string | Jlenia Monfregola | |
| Jing He | |||
| Jennifer L Johnson | |||
| Kersi Pestonjamasp | |||
| P2860 | cites work | Guidelines for the use and interpretation of assays for monitoring autophagy | Q21996341 |
| Syntaxin 7 and VAMP-7 are soluble N-ethylmaleimide-sensitive factor attachment protein receptors required for late endosome-lysosome and homotypic lysosome fusion in alveolar macrophages | Q22254581 | ||
| Cystinosis | Q24262959 | ||
| The targeting of cystinosin to the lysosomal membrane requires a tyrosine-based signal and a novel sorting motif | Q24290722 | ||
| A receptor for the selective uptake and degradation of proteins by lysosomes | Q24319797 | ||
| A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis | Q24322985 | ||
| Cathepsin A regulates chaperone-mediated autophagy through cleavage of the lysosomal receptor | Q24337837 | ||
| Activation of chaperone-mediated autophagy during oxidative stress | Q24562065 | ||
| LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing | Q24597817 | ||
| Chaperone-mediated autophagy: a unique way to enter the lysosome world | Q24628159 | ||
| In vivo analysis of autophagy in response to nutrient starvation using transgenic mice expressing a fluorescent autophagosome marker | Q24633015 | ||
| mTOR: from growth signal integration to cancer, diabetes and ageing | Q24633662 | ||
| The chaperone-mediated autophagy receptor organizes in dynamic protein complexes at the lysosomal membrane | Q24646454 | ||
| Lysosome membrane lipid microdomains: novel regulators of chaperone-mediated autophagy | Q24676280 | ||
| Autophagy in the Pathogenesis of Disease | Q27860558 | ||
| Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter | Q27937730 | ||
| CTNS mutations in patients with cystinosis | Q28138498 | ||
| A block of autophagy in lysosomal storage disorders | Q28252016 | ||
| Unique properties of lamp2a compared to other lamp2 isoforms | Q28569157 | ||
| Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease | Q28748170 | ||
| Regulation mechanisms and signaling pathways of autophagy | Q29547416 | ||
| Autophagy: from phenomenology to molecular understanding in less than a decade | Q29614174 | ||
| Dissection of the autophagosome maturation process by a novel reporter protein, tandem fluorescent-tagged LC3 | Q29614176 | ||
| Activation of focal adhesion kinase by Salmonella suppresses autophagy via an Akt/mTOR signaling pathway and promotes bacterial survival in macrophages | Q30412570 | ||
| Upregulation of the Rab27a-dependent trafficking and secretory mechanisms improves lysosomal transport, alleviates endoplasmic reticulum stress, and reduces lysosome overload in cystinosis | Q30541728 | ||
| Mitochondrial autophagy promotes cellular injury in nephropathic cystinosis | Q33711275 | ||
| Analysis of CTNS gene transcripts in nephropathic cystinosis | Q33866784 | ||
| Lysosomal fusion and SNARE function are impaired by cholesterol accumulation in lysosomal storage disorders | Q34140173 | ||
| Inhibitory effect of dietary lipids on chaperone-mediated autophagy | Q34254155 | ||
| Signals from the lysosome: a control centre for cellular clearance and energy metabolism | Q34340780 | ||
| Consequences of the selective blockage of chaperone-mediated autophagy | Q34596028 | ||
| Lysosome biogenesis and lysosomal membrane proteins: trafficking meets function | Q34996827 | ||
| Chaperone-mediated autophagy | Q35107699 | ||
| Oxidative stress in cystinosis patients | Q35791769 | ||
| Steady-state pharmacokinetics and pharmacodynamics of cysteamine bitartrate in paediatric nephropathic cystinosis patients | Q35825821 | ||
| The cell biology of lysosomal storage disorders | Q35825859 | ||
| Cysteamine therapy: a treatment for cystinosis, not a cure | Q36068161 | ||
| Substrate reduction therapy for lysosomal storage diseases | Q36128571 | ||
| Chaperone-mediated autophagy in aging and disease | Q36510519 | ||
| Constitutive activation of chaperone-mediated autophagy in cells with impaired macroautophagy | Q36631143 | ||
| Pantethine and cystamine deplete cystine from cystinotic fibroblasts via efflux of cysteamine-cysteine mixed disulfide | Q36992736 | ||
| Nephropathic cystinosis: late complications of a multisystemic disease | Q37002855 | ||
| Selective autophagy: talking with the UPS. | Q38109587 | ||
| Lysosomal turnover, but not a cellular level, of endogenous LC3 is a marker for autophagy | Q40250473 | ||
| Regional deficiencies in chaperone-mediated autophagy underlie α-synuclein aggregation and neurodegeneration | Q42078692 | ||
| Regulation of lamp2a levels in the lysosomal membrane | Q42831940 | ||
| Effect of experimental treatment on housekeeping gene expression: validation by real-time, quantitative RT-PCR. | Q42833946 | ||
| Renal phenotype of the cystinosis mouse model is dependent upon genetic background | Q43257283 | ||
| Autophagy: evolutionary and pathophysiological insights | Q43749203 | ||
| hVps41 and VAMP7 function in direct TGN to late endosome transport of lysosomal membrane proteins | Q44294106 | ||
| N-acetyl-cysteine is associated to renal function improvement in patients with nephropathic cystinosis. | Q46027363 | ||
| Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis | Q46665497 | ||
| Monitoring autophagic degradation of p62/SQSTM1. | Q50603624 | ||
| Chaperone-mediated autophagy. | Q55130784 | ||
| Report of a Brazilian multicenter study on nephropathic cystinosis | Q56780847 | ||
| Nephropathic cystinosis in adults: natural history and effects of oral cysteamine therapy | Q64045497 | ||
| Biosynthesis, glycosylation, movement through the Golgi system, and transport to lysosomes by an N-linked carbohydrate-independent mechanism of three lysosomal integral membrane proteins | Q69664008 | ||
| The intralysosomal pH in cultured human skin fibroblasts in relation to cystine accumulation in patients with cystinosis | Q71094953 | ||
| Lysosomal storage diseases as disorders of autophagy | Q79827971 | ||
| Long-term treatment of infantile nephropathic cystinosis with cysteamine | Q93637313 | ||
| P433 | issue | 2 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | lysosomal storage disease | Q675010 |
| autophagy | Q288322 | ||
| molecular chaperones | Q422496 | ||
| P304 | page(s) | 158-174 | |
| P577 | publication date | 2015-01-12 | |
| P1433 | published in | EMBO Molecular Medicine | Q15817279 |
| P1476 | title | Impairment of chaperone-mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis | |
| P478 | volume | 7 |
| Q96952478 | A substrate-specific mTORC1 pathway underlies Birt-Hogg-Dubé syndrome |
| Q24701804 | Apoptosis, autophagy and unfolded protein response pathways in Arbovirus replication and pathogenesis |
| Q93203407 | Autonomic Nervous System Dysregulation in Monozygous Twins With Nephropathic Cystinosis |
| Q64232167 | Autophagy Intertwines with Different Diseases-Recent Strategies for Therapeutic Approaches |
| Q38542636 | Autophagy in renal diseases |
| Q37742983 | CMA down-regulates p53 expression through degradation of HMGB1 protein to inhibit irradiation-triggered apoptosis in hepatocellular carcinoma |
| Q61803757 | Chaperone-Mediated Autophagy Upregulation Rescues Megalin Expression and Localization in Cystinotic Proximal Tubule Cells |
| Q47287498 | Chaperone-mediated autophagy and endosomal microautophagy: joint by a chaperone |
| Q36810948 | Chaperone-mediated autophagy regulates proliferation by targeting RND3 in gastric cancer |
| Q92497107 | Cross-regulation of defective endolysosome trafficking and enhanced autophagy through TFEB in UNC13D deficiency |
| Q46864655 | Cystinosin, the small GTPase Rab11, and the Rab7 effector RILP regulate intracellular trafficking of the chaperone-mediated autophagy receptor LAMP2A. |
| Q30838649 | Cystinosis (ctns) zebrafish mutant shows pronephric glomerular and tubular dysfunction. |
| Q92366681 | Dysregulated DNA methylation of GLA gene was associated with dysfunction of autophagy |
| Q99711433 | Editorial: Autophagy in Endocrine-Metabolic Diseases Associated With Aging |
| Q39053014 | Functional and Molecular Insights of Hydrogen Sulfide Signaling and Protein Sulfhydration |
| Q57821047 | Genes involved in the regulation of different types of autophagy and their participation in cancer pathogenesis |
| Q22676705 | Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) |
| Q51733341 | Impact of atypical mitochondrial cyclic-AMP level in nephropathic cystinosis. |
| Q48263283 | Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney. |
| Q47140730 | Impaired p65 degradation by decreased chaperone-mediated autophagy activity facilitates epithelial-to-mesenchymal transition |
| Q64923813 | Interaction between galectin-3 and cystinosin uncovers a pathogenic role of inflammation in kidney involvement of cystinosis. |
| Q41280083 | M2 tumor-associated macrophages produce interleukin-17 to suppress oxaliplatin-induced apoptosis in hepatocellular carcinoma |
| Q92327164 | Mitochondrial Dynamics of Proximal Tubular Epithelial Cells in Nephropathic Cystinosis |
| Q47301133 | Molecular control of chaperone-mediated autophagy |
| Q64042540 | Multidisciplinary approach for patients with nephropathic cystinosis: model for care in a rare and chronic renal disease |
| Q30710923 | Munc13-4 Is a Rab11-binding Protein That Regulates Rab11-positive Vesicle Trafficking and Docking at the Plasma Membrane. |
| Q27305785 | Munc13-4 interacts with syntaxin 7 and regulates late endosomal maturation, endosomal signaling, and TLR9-initiated cellular responses |
| Q52624159 | Nephropathic Cystinosis: Symptoms, Treatment, and Perspectives of a Systemic Disease. |
| Q90239959 | Protection of Cystinotic Mice by Kidney-Specific Megalin Ablation Supports an Endocytosis-Based Mechanism for Nephropathic Cystinosis Progression |
| Q38998708 | Receptor-Mediated Endocytosis in the Proximal Tubule |
| Q94595417 | Regulation of TFEB activity and its potential as a therapeutic target against kidney diseases |
| Q37072011 | Role of chaperone-mediated autophagy in metabolism |
| Q52329462 | The coming of age of chaperone-mediated autophagy. |
| Q38781097 | The renal Fanconi syndrome in cystinosis: pathogenic insights and therapeutic perspectives |
| Q90541471 | Use of Human Induced Pluripotent Stem Cells and Kidney Organoids To Develop a Cysteamine/mTOR Inhibition Combination Therapy for Cystinosis |
| Q58770831 | mTOR-dependent phosphorylation controls TFEB nuclear export |
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