Related references
Note: Only part of the references are listed.The Degree of Aminoacidemia after Dairy Protein Ingestion Does Not Modulate the Postexercise Anabolic Response in Young Men: A Randomized Controlled Trial
Alex H. Chan et al.
JOURNAL OF NUTRITION (2019)
Quantification of cystine in human renal proximal tubule cells using liquid chromatography-tandem mass spectrometry
Amer Jamalpoor et al.
BIOMEDICAL CHROMATOGRAPHY (2018)
Everolimus is a new anti-cancer molecule: Metabolic side effects as lipid disorders and hyperglycemia
L. Morviducci et al.
DIABETES RESEARCH AND CLINICAL PRACTICE (2018)
Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney
Beatrice Paola Festa et al.
NATURE COMMUNICATIONS (2018)
A Simple Bioreactor-Based Method to Generate Kidney Organoids from Pluripotent Stem Cells
Aneta Przepiorski et al.
STEM CELL REPORTS (2018)
On the Role of Basal Autophagy in Adult Neural Stem Cells and Neurogenesis
Lucia Casares-Crespo et al.
FRONTIERS IN CELLULAR NEUROSCIENCE (2018)
The renal Fanconi syndrome in cystinosis: pathogenic insights and therapeutic perspectives
Stephanie Cherqui et al.
NATURE REVIEWS NEPHROLOGY (2017)
Dysregulation of autophagy as a common mechanism in lysosomal storage diseases
Elena Seranova et al.
SIGNALLING MECHANISMS IN AUTOPHAGY (2017)
New insights into autophagosome-lysosome fusion
Shuhei Nakamura et al.
JOURNAL OF CELL SCIENCE (2017)
mTORC1 hyperactivation arrests bone growth in lysosomal storage disorders by suppressing autophagy
Rosa Bartolomeo et al.
JOURNAL OF CLINICAL INVESTIGATION (2017)
Cystinosis (ctns) zebrafish mutant shows pronephric glomerular and tubular dysfunction
Mohamed A. Elmonem et al.
SCIENTIFIC REPORTS (2017)
Basal autophagy prevents autoactivation or enhancement of inflammatory signals by targeting monomeric MyD88
Takeshi Into et al.
SCIENTIFIC REPORTS (2017)
Detection and Clearance of Damaged Lysosomes by the Endo-Lysosomal Damage Response and Lysophagy
Chrisovalantis Papadopoulos et al.
CURRENT BIOLOGY (2017)
Acute resistance exercise induces Sestrin2 phosphorylation and p62 dephosphorylation in human skeletal muscle
Nina Zeng et al.
PHYSIOLOGICAL REPORTS (2017)
Everolimus immunosuppression for renal protection, reduction of allograft vasculopathy and prevention of allograft rejection in de-novo heart transplant recipients: could we have it all?
Einar Gude et al.
CURRENT OPINION IN ORGAN TRANSPLANTATION (2017)
Cystinosin is a Component of the Vacuolar H+-ATPase-Ragulator-Rag Complex Controlling Mammalian Target of Rapamycin Complex 1 Signaling
Zuzanna Andrzejewska et al.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY (2016)
Long and short (timeframe) of endoplasmic reticulum stress-induced cell death
Hyung Don Ryoo
FEBS JOURNAL (2016)
Altered mTOR signalling in nephropathic cystinosis
Ekaterina A. Ivanova et al.
JOURNAL OF INHERITED METABOLIC DISEASE (2016)
Niemann-Pick type C2 deficiency impairs autophagy-lysosomal activity, mitochondrial function, and TLR signaling in adipocytes
Hong Guo et al.
JOURNAL OF LIPID RESEARCH (2016)
Activation of the transcription factor EB rescues lysosomal abnormalities in cystinotic kidney cells
Laura R. Rega et al.
KIDNEY INTERNATIONAL (2016)
Bafilomycin A1 disrupts autophagic flux by inhibiting both V-ATPase-dependent acidification and Ca-P60A/SERCA-dependent autophagosome-lysosome fusion
Caroline Mauvezin et al.
AUTOPHAGY (2015)
Impairment of chaperone-mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis
Gennaro Napolitano et al.
EMBO MOLECULAR MEDICINE (2015)
Altered TFEB-mediated lysosomal biogenesis in Gaucher disease iPSC-derived neuronal cells
Ola Awad et al.
HUMAN MOLECULAR GENETICS (2015)
Redox control of protein degradation
Marta Pajares et al.
REDOX BIOLOGY (2015)
Endo-Lysosomal Dysfunction in Human Proximal Tubular Epithelial Cells Deficient for Lysosomal Cystine Transporter Cystinosin
Ekaterina A. Ivanova et al.
PLOS ONE (2015)
The swan-neck lesion: proximal tubular adaptation to oxidative stress in nephropathic cystinosis
Carolina I. Galarreta et al.
AMERICAN JOURNAL OF PHYSIOLOGY-RENAL PHYSIOLOGY (2015)
Cas-OFFinder: a fast and versatile algorithm that searches for potential off-target sites of Cas9 RNA-guided endonucleases
Sangsu Bae et al.
BIOINFORMATICS (2014)
Dedifferentiation and aberrations of the endolysosomal compartment characterize the early stage of nephropathic cystinosis
Claudia Raggi et al.
HUMAN MOLECULAR GENETICS (2014)
Time Course of Pathogenic and Adaptation Mechanisms in Cystinotic Mouse Kidneys
Heloise P. Gaide Chevronnay et al.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY (2014)
Nephropathic cystinosis: an international consensus document
Francesco Emma et al.
NEPHROLOGY DIALYSIS TRANSPLANTATION (2014)
A Modified Method for Implantation of Pluripotent Stem Cells Under the Rodent Kidney Capsule
Fuli Zhu et al.
STEM CELLS AND DEVELOPMENT (2014)
Genetic and Chemical Correction of Cholesterol Accumulation and Impaired Autophagy in Hepatic and Neural Cells Derived from Niemann-Pick Type C Patient-Specific iPS Cells
Dorothea Maetzel et al.
STEM CELL REPORTS (2014)
Basal Autophagy Is Required for the Efficient Catabolism of Sialyloligosaccharides
Junichi Seino et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2013)
Integration-Free Induced Pluripotent Stem Cells Model Genetic and Neural Developmental Features of Down Syndrome Etiology
James A. Briggs et al.
STEM CELLS (2013)
Spatiotemporally controlled induction of autophagy-mediated lysosome turnover
Yu-Hsien Hung et al.
NATURE COMMUNICATIONS (2013)
Impaired Autophagy in the Lipid-Storage Disorder Niemann-Pick Type C1 Disease
Sovan Sarkar et al.
CELL REPORTS (2013)
Autophagy in lysosomal storage disorders
Andrew P. Lieberman et al.
AUTOPHAGY (2012)
Autophagy plays a critical role in kidney tubule maintenance, aging and ischemia-reperfusion injury
Shuya Liu et al.
AUTOPHAGY (2012)
mTOR inhibitors in cancer therapy
Yekaterina Y. Zaytseva et al.
CANCER LETTERS (2012)
A lysosome-to-nucleus signalling mechanism senses and regulates the lysosome via mTOR and TFEB
Carmine Settembre et al.
EMBO JOURNAL (2012)
Mammalian proapoptotic factor ChaC1 and its homologues function as γ-glutamyl cyclotransferases acting specifically on glutathione
Akhilesh Kumar et al.
EMBO REPORTS (2012)
Autophagy in proximal tubules protects against acute kidney injury
Man Jiang et al.
KIDNEY INTERNATIONAL (2012)
Amino acids and mTORC1: from lysosomes to disease
Alejo Efeyan et al.
TRENDS IN MOLECULAR MEDICINE (2012)
The protective role of autophagy against aging and acute ischemic injury in kidney proximal tubular cells
Yoshitaka Isaka et al.
AUTOPHAGY (2011)
Cysteamine restores glutathione redox status in cultured cystinotic proximal tubular epithelial cells
Martijn J. Wilmer et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2011)
Autophagy-mediated chemosensitization by cysteamine in cancer cells
Xiao-Mei Wan et al.
INTERNATIONAL JOURNAL OF CANCER (2011)
Autophagy Protects the Proximal Tubule from Degeneration and Acute Ischemic Injury
Tomonori Kimura et al.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY (2011)
Energy Metabolism in Human Pluripotent Stem Cells and Their Differentiated Counterparts
Sandra Varum et al.
PLOS ONE (2011)
Ragulator-Rag Complex Targets mTORC1 to the Lysosomal Surface and Is Necessary for Its Activation by Amino Acids
Yasemin Sancak et al.
CELL (2010)
Antioxidants can inhibit basal autophagy and enhance neurodegeneration in models of polyglutamine disease
Benjamin R. Underwood et al.
HUMAN MOLECULAR GENETICS (2010)
Mitochondrial Autophagy Promotes Cellular Injury in Nephropathic Cystinosis
Poonam Sansanwal et al.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY (2010)
Renal phenotype of the cystinosis mouse model is dependent upon genetic background
Nathalie Nevo et al.
NEPHROLOGY DIALYSIS TRANSPLANTATION (2010)
Neurological impairment in nephropathic cystinosis: motor coordination deficits
Doris A. Trauner et al.
PEDIATRIC NEPHROLOGY (2010)
The pathogenesis of cystinosis: mechanisms beyond cystine accumulation
Martijn J. Wilmer et al.
AMERICAN JOURNAL OF PHYSIOLOGY-RENAL PHYSIOLOGY (2010)
Everolimus Represents an Advance in Immunosuppression for Patients Who Have Developed Cancer After Renal Transplantation
E. Gonzalez et al.
TRANSPLANTATION PROCEEDINGS (2009)
Immunoregulatory functions of mTOR inhibition
Angus W. Thomson et al.
NATURE REVIEWS IMMUNOLOGY (2009)
Urinary protein excretion pattern and renal expression of megalin and cubilin in nephropathic cystinosis
Martijn J. Wilmer et al.
AMERICAN JOURNAL OF KIDNEY DISEASES (2008)
Autophagy in the pathogenesis of disease
Beth Levine et al.
CELL (2008)
Suppression of autophagy in skeletal muscle uncovers the accumulation of ubiquitinated proteins and their potential role in muscle damage in Pompe disease
Nina Raben et al.
HUMAN MOLECULAR GENETICS (2008)
Autophagosome formation from membrane compartments enriched in phosphatidylinositol 3-phosphate and dynamically connected to the endoplasmic reticulum
Elizabeth L. Axe et al.
JOURNAL OF CELL BIOLOGY (2008)
A protocol describing the use of a recombinant protein-based, animal product-free medium (APEL) for human embryonic stem cell differentiation as spin embryoid bodies
Elizabeth S. Ng et al.
NATURE PROTOCOLS (2008)
The Rag GTPases bind raptor and mediate amino acid signaling to mTORC1
Yasemin Sancak et al.
SCIENCE (2008)
Dissection of the autophagosome maturation process by a novel reporter protein, tandem fluorescent-tagged LC3
Shunsuke Kimura et al.
AUTOPHAGY (2007)
Nephropathic cystinosis in adults: Natural history and effects of oral cysteamine therapy
William A. Gahl et al.
ANNALS OF INTERNAL MEDICINE (2007)
Autophagosome-lysosome fusion depends on th pH in acidic compartments in CHO cells
Akinori Kawai et al.
AUTOPHAGY (2007)
Increased apoptosis in cystinotic fibroblasts and renal proximal tubule epithelial cells results from cysteinylation of protein kinase Cδ
Margaret A. Park et al.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY (2006)
Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells
GF Laube et al.
PEDIATRIC NEPHROLOGY (2006)
Impaired activity of the γ-glutamyl cycle in nephropathic cystinosis fibroblasts
L Mannucci et al.
PEDIATRIC RESEARCH (2006)
Strict cysteamine dose regimen is required to prevent nocturnal cystine accumulation in cystinosis
EN Levtchenko et al.
PEDIATRIC NEPHROLOGY (2006)
Elevated oxidized glutathione in cystinotic proximal tubular epithelial cells
MJG Wilmer et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2005)
Altered status of glutathione and its metabolites in eystinotic cells
E Levtchenko et al.
NEPHROLOGY DIALYSIS TRANSPLANTATION (2005)
Effects of simian virus 40 large and small tumor antigens on mammalian target of rapamycin signaling: Small tumor antigen mediates hypophosphorylation of eIF4E-binding protein 1 late in infection
YJ Yu et al.
JOURNAL OF VIROLOGY (2005)
Impairment of starvation-induced and constitutive autophagy in Atg7-deficient mice
M Komatsu et al.
JOURNAL OF CELL BIOLOGY (2005)
TRB3, a novel ER stress-inducible gene, is induced via ATF4-CHOP pathway and is involved in cell death
N Ohoka et al.
EMBO JOURNAL (2005)
Molecular pathogenesis of cystinosis:: effect of CTNS mutations on the transport activity and subcellular localization of cystinosin
V Kalatzis et al.
HUMAN MOLECULAR GENETICS (2004)
Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines
M Chol et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2004)
Susceptibility of lysosomes to rupture is a determinant for plasma membrane disruption in tumor necrosis factor alpha-induced cell death
K Ono et al.
MOLECULAR AND CELLULAR BIOLOGY (2003)
Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells
M Park et al.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY (2002)
Intralysosomal cystine accumulation in mice lacking cystinosin, the protein defective in cystinosis
S Cherqui et al.
MOLECULAR AND CELLULAR BIOLOGY (2002)
Cystinosis
WA Gahl et al.
NEW ENGLAND JOURNAL OF MEDICINE (2002)
Analysis of relative gene expression data using real-time quantitative PCR and the 2-ΔΔCT method
KJ Livak et al.
METHODS (2001)
Cystinosin, the protein defective in cystinosis, is a H+-driven lysosomal cystine transporter
V Kalatzis et al.
EMBO JOURNAL (2001)
Share Paper
