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2 ants of mineral homeostasis in patients with nephropathic cystinosis across the predialysis CKD spect
3 and Drug Administration-approved therapy for nephropathic cystinosis also postulated to enhance gluta
4 t in three children and three adults who had nephropathic cystinosis and corneal cystine depositions.
5 e, and thus has potential as a treatment for nephropathic cystinosis and other renal lysosomal storag
6 e megalin/LRP2 pathway in the progression of nephropathic cystinosis and provide a proof of concept f
7 votal factor in the cell injury mechanism of nephropathic cystinosis and provide evidence linking cel
8 dysfunction and progressive renal failure in nephropathic cystinosis are largely unclear, and increas
9 nt cystine accumulation slows progression of nephropathic cystinosis but it is a demanding treatment
13 normal range, thus protecting patients with nephropathic cystinosis from elevations of FGF23 during
22 s unknown if persistent phosphate wasting in nephropathic cystinosis is associated with a biochemical
23 analyses demonstrated lower FGF23 levels in nephropathic cystinosis participants at all CKD stages w
26 agement of primary hyperoxalurias as well as nephropathic cystinosis provide important general inform
27 This suggests the involvement of pathways in nephropathic cystinosis that are unrelated to lysosomal
32 n and crystals at acidic pH in patients with nephropathic cystinosis, a rare lysosomal storage diseas
34 mutations in the CTNS gene, is a hallmark of nephropathic cystinosis, but the role of these crystals
36 In kidney biopsy samples from patients with nephropathic cystinosis, clusterin protein expression wa
37 te, an antioxidant therapy for patients with nephropathic cystinosis, in a mouse model of unilateral
38 hogenic mutations in patients with infantile nephropathic cystinosis, including a common, approximate
40 s study, we screened patients with infantile nephropathic cystinosis, those with late-onset cystinosi
41 e of pathogenic and adaptation mechanisms of nephropathic cystinosis, we defined the onset of Fanconi
42 molecular and cellular mechanisms underlying nephropathic cystinosis, which exhibits generalized prox
43 functional evidence of abnormal mitophagy in nephropathic cystinosis, which may contribute to the ren
56 It is here reported that in both normal and nephropathic cystinotic fibroblasts and cultured renal p
57 thdrawal causes an apoptotic rate of 8.7% in nephropathic cystinotic fibroblasts, compared with 6.1%
58 sure induced apoptosis in 18.1% and 17.4% of nephropathic cystinotic fibroblasts, respectively, versu