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1 ffects on plasma testosterone levels in male nephropathic animals.
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
10                             One patient with nephropathic cystinosis carried a -295 G-->C substitutio
11                                    Untreated nephropathic cystinosis causes extensive morbidity and d
12                                              Nephropathic cystinosis CKD patients have mineral abnorm
13  normal range, thus protecting patients with nephropathic cystinosis from elevations of FGF23 during
14                                Patients with nephropathic cystinosis had significantly lower percent
15                           The full burden of nephropathic cystinosis in adulthood and the effects of
16                                              Nephropathic cystinosis is a lethal disorder of lysosoma
17                                              Nephropathic cystinosis is a rare disease secondary to r
18                                              Nephropathic cystinosis is a rare inherited lysosomal st
19                                    Infantile nephropathic cystinosis is a rare, autosomal recessive d
20                                              Nephropathic cystinosis is an autosomal recessive disord
21                                              Nephropathic cystinosis is an autosomal recessive lysoso
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
24      Mutation analysis of 108 American-based nephropathic cystinosis patients revealed that 48 patien
25           The rare lysosomal storage disease nephropathic cystinosis presents with renal Fanconi synd
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
28  effected in patients with CKD stemming from nephropathic cystinosis versus other causes.
29            Out of 25 patients with infantile nephropathic cystinosis, 12 have two severely truncating
30                           Of 100 adults with nephropathic cystinosis, 92 had received a renal allogra
31                                              Nephropathic cystinosis, a hereditary lysosomal storage
32 n and crystals at acidic pH in patients with nephropathic cystinosis, a rare lysosomal storage diseas
33                                              Nephropathic cystinosis, an autosomal recessive disorder
34 mutations in the CTNS gene, is a hallmark of nephropathic cystinosis, but the role of these crystals
35                                              Nephropathic cystinosis, characterized by accumulation o
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
39                   The most severe phenotype, nephropathic cystinosis, manifests during the first mont
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
44          The study included 50 patients with nephropathic cystinosis-related CDK and 97 with CKD from
45 omarker for use in therapeutic monitoring of nephropathic cystinosis.
46 epithelial cells obtained from patients with nephropathic cystinosis.
47 ntified clusterin as potentially involved in nephropathic cystinosis.
48 oni syndrome and progressive renal injury in nephropathic cystinosis.
49  for cystine crystals in the pathogenesis of nephropathic cystinosis.
50 ched patients, with causes of CKD other than nephropathic cystinosis.
51 acuoles and fewer mitochondria (P < 0.02) in nephropathic cystinosis.
52  restore the function of altered pathways in nephropathic cystinosis.
53 optimization of cystine depletion therapy in nephropathic cystinosis.
54 et on kidney disease of two animal models of nephropathic cystinosis.
55 ptotic rate is increased to the rate seen in nephropathic cystinotic cells.
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
59                                           In nephropathic cystinotic fibroblasts, the rate of apoptos
60  in many tissues and hence contribute to the nephropathic cystinotic phenotype.
61 of the disease was milder than the infantile nephropathic form.
62 y of Hope conference, all with the infantile nephropathic form.
63  with three clinical variants of cystinosis: Nephropathic, intermediate, and ocular.
64 nal clearable gold NPs (AuNPs) in normal and nephropathic kidneys.
65      How lysosomal cystine causes the lethal nephropathic phenotype is unknown.
66 and significantly more autophagosomes in the nephropathic variant.