ArticlePediatric nephrology (Berlin, Germany)2012
p62/SQSTM1 prominently accumulates in renal proximal tubules in nephropathic cystinosis.
Article in Pediatric nephrology (Berlin, Germany), 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 42 citations in OpenAlex.
- Cystinosis and Cellular Energy Failure: Mitochondria at the Crossroads.International journal of molecular sciences · 2026Review
- Mechanisms of Xuefu Zhuyu decoction in treating diabetic kidney disease-induced renal fibrosis: UPLC-Q/TOF-MS, network pharmacology, and experimental validation.The Korean journal of physiology & pharmacology : official journal of the Korean Physiological Society and the Korean Society of Pharmacology · 2025Article
- Article
- Ketogenic Diet and Progression of Kidney Disease in Animal Models of Nephropathic Cystinosis.Journal of the American Society of Nephrology : JASN · 2024Article
- Review
- Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies.Cells · 2022Review
- Article
- Review
- Cysteamine-bicalutamide combination therapy corrects proximal tubule phenotype in cystinosis.EMBO molecular medicine · 2021Article
- Ferroptosis and Its Modulation by Autophagy in Light of the Pathogenesis of Lysosomal Storage Diseases.Cells · 2021Review
- Cell-Based Phenotypic Drug Screening Identifies Luteolin as Candidate Therapeutic for Nephropathic Cystinosis.Journal of the American Society of Nephrology : JASN · 2020Article
- The aminoglycoside geneticin permits translational readthrough of the CTNS W138X nonsense mutation in fibroblasts from patients with nephropathic cystinosis.Pediatric nephrology (Berlin, Germany) · 2019Article
- Dynamic expression and roles of sequestome‑1/p62 in LPS‑induced acute kidney injury in mice.Molecular medicine reports · 2018Article
- Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney.Nature communications · 2018Article
- Autophagy in renal diseases.Pediatric nephrology (Berlin, Germany) · 2016Review
- Altered mTOR signalling in nephropathic cystinosis.Journal of inherited metabolic disease · 2016Article
- Autophagy in Chronic Kidney Diseases.Kidney diseases (Basel, Switzerland) · 2016Review
- The crucial role of vitamin C and its transporter (SVCT2) in bone marrow stromal cell autophagy and apoptosis.Stem cell research · 2015Article
- Inhibition of intracellular clusterin attenuates cell death in nephropathic cystinosis.Journal of the American Society of Nephrology : JASN · 2015Article
- Lysosome dysfunction in the pathogenesis of kidney diseases.Pediatric nephrology (Berlin, Germany) · 2014Review
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
backgroundNephropathic cystinosis, a lysosomal storage disorder, is associated with generalized proximal tubular dysfunction and progressive renal failure. The underlying molecular and cellular mechanisms leading to renal tubular injury remain largely unknown. Abnormal induction of autophagy has been shown in cystinosis. We have studied the autophagic flux in cystinosis by evaluating autophagy-specific substrates.
methodsLC3 and p62 expression was evaluated by (1) immunohistochemistry performed on kidney biopsies obtained from four nephropathic cystinosis patients, four patients with renal injury due to causes other than cystinosis, and four normal kidney tissues and (2) fluorescence imaging in cultured renal proximal tubular epithelial (RPTE) cells obtained from four nephropathic cystinosis patients and two lots of normal primary RPTE cells, both in basal and starvation conditions. p62 expression was also corroborated by western blot analysis in RPTE cells.
resultsThere was a significant buildup of p62 protein in patients with nephropathic cystinosis, specifically in the proximal tubules in kidney biopsies and RPTE cells (p = 0.0004), and the accumulation was further enhanced upon starvation. Cystinotic RPTE cells exhibited a significant co-localization of p62 with LC3.
conclusionsOur findings indicate a potential block in the autophagic flux in cystinosis, thus providing key insights into the underlying mechanisms of tubular injury in cystinosis.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.