| Q33348982 | A futile cycle, formed between two ATP-dependant gamma-glutamyl cycle enzymes, gamma-glutamyl cysteine synthetase and 5-oxoprolinase: the cause of cellular ATP depletion in nephrotic cystinosis? |
| Q38083646 | A patient with cystinosis presenting like bartter syndrome and review of literature |
| Q71602375 | A simple luciferase assay to measure atp levels in small numbers of cells using a fluorescent plate reader |
| Q36827084 | Ablation of dihydroceramide desaturase 1, a therapeutic target for the treatment of metabolic diseases, simultaneously stimulates anabolic and catabolic signaling |
| Q37426990 | Acetaminophen toxicity and 5-oxoproline (pyroglutamic acid): a tale of two cycles, one an ATP-depleting futile cycle and the other a useful cycle |
| Q52705283 | Albumin uptake in OK cells exposed to rotenone: a model for studying the effects of mitochondrial dysfunction on endocytosis in the proximal tubule? |
| Q40802929 | Aminoglycoside-induced Fanconi's syndrome |
| Q84461134 | Caspase-4 may play a role in loss of proximal tubules and renal injury in nephropathic cystinosis |
| Q42167483 | Cysteamine prevents inhibition of adenylate kinase caused by cystine in rat brain cortex |
| Q43290415 | Cysteamine prevents inhibition of adenylate kinase caused by cystine in rat brain cortex |
| Q46640050 | Cysteamine prevents inhibition of thiol-containing enzymes caused by cystine or cystine dimethylester loading in rat brain cortex |
| Q32872002 | Cystine accumulation attenuates insulin release from the pancreatic β-cell due to elevated oxidative stress and decreased ATP levels |
| Q46312904 | Cystinosis, Fanconi syndrome, and odontogenic cysts |
| Q47645665 | Cystinosis. A clinicopathological conference. 'From toddlers to twenties and beyond' Adult-Paediatric Nephrology Interface Meeting, Manchester 2001 |
| Q24302197 | Decreased intracellular ATP content and intact mitochondrial energy generating capacity in human cystinotic fibroblasts |
| Q27930559 | Disruption of a cystine transporter downregulates expression of genes involved in sulfur regulation and cellular respiration |
| Q45207707 | Exfoliated human proximal tubular cells: a model of cystinosis and Fanconi syndrome |
| Q42491489 | Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells |
| Q49287935 | Hypercalciuria and ultrasound abnormalities in children with cystinosis |
| Q46913688 | Impaired activity of the gamma-glutamyl cycle in nephropathic cystinosis fibroblasts |
| Q80186614 | Infantile nephropathic cystinosis |
| Q34431244 | Inhibition of intracellular clusterin attenuates cell death in nephropathic cystinosis |
| Q28591791 | Intralysosomal cystine accumulation in mice lacking cystinosin, the protein defective in cystinosis |
| Q46023359 | Kidney preservation by bone marrow cell transplantation in hereditary nephropathy. |
| Q38161818 | Lysosome dysfunction in the pathogenesis of kidney diseases. |
| Q49073643 | Management dilemmas in pediatric nephrology: Cystinosis. |
| Q33711275 | Mitochondrial autophagy promotes cellular injury in nephropathic cystinosis |
| Q46505867 | Mitochondrial complex V expression and activity in cystinotic fibroblasts |
| Q35088312 | New aspects of the pathogenesis of cystinosis. |
| Q80396751 | Promotion of oxidative stress in kidney of rats loaded with cystine dimethyl ester |
| Q42413378 | Renal Fanconi Syndrome Secondary to Deferasirox: Where There Is Smoke There Is Fire |
| Q41321223 | Renal proximal tubular epithelium from patients with nephropathic cystinosis: immortalized cell lines as in vitro model systems |
| Q40774671 | The cellular basis of Fanconi syndrome |
| Q37786450 | The pathogenesis of cystinosis: mechanisms beyond cystine accumulation |
| Q53440988 | The reduced expression of proximal tubular transporters in acquired Fanconi syndrome with κ light chain deposition. |
| Q38781097 | The renal Fanconi syndrome in cystinosis: pathogenic insights and therapeutic perspectives |
| Q42167144 | The use of CDME in cystinosis research |
| Q33901252 | Thiol-disulfide effects on hepatic glutathione transport. Studies in cultured rat hepatocytes and perfused livers |
| Q54986468 | Thiol/disulfide status regulates the activity of thiol-containing kinases related to energy homeostasis in rat kidney. |
| Q34444456 | Treatment of corneal cystine crystal accumulation in patients with cystinosis |
| Q90541471 | Use of Human Induced Pluripotent Stem Cells and Kidney Organoids To Develop a Cysteamine/mTOR Inhibition Combination Therapy for Cystinosis |
| Q42508251 | p62/SQSTM1 prominently accumulates in renal proximal tubules in nephropathic cystinosis |