Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies.
Mohamed A ElmonemKoenraad R P VeysGiusi PrencipePublished in: Cells (2022)
The activation of several inflammatory pathways has recently been documented in patients and different cellular and animal models of nephropathic cystinosis. Upregulated inflammatory signals interact with many pathogenic aspects of the disease, such as enhanced oxidative stress, abnormal autophagy, inflammatory cell recruitment, enhanced cell death, and tissue fibrosis. Cysteamine, the only approved specific therapy for cystinosis, ameliorates many but not all pathogenic aspects of the disease. In the current review, we summarize the inflammatory mechanisms involved in cystinosis and their potential impact on the disease pathogenesis and progression. We further elaborate on the crosstalk between inflammation, autophagy, and apoptosis, and discuss the potential of experimental drugs for suppressing the inflammatory signals in cystinosis.
Keyphrases
- oxidative stress
- cell death
- diabetic rats
- dna damage
- ischemia reperfusion injury
- induced apoptosis
- endoplasmic reticulum stress
- signaling pathway
- end stage renal disease
- ejection fraction
- risk assessment
- human health
- peritoneal dialysis
- newly diagnosed
- mesenchymal stem cells
- cell therapy
- climate change
- prognostic factors
- patient reported outcomes
- bone marrow
- mouse model
- cell proliferation