Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies
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,...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-01-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/11/2/190 |
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| author | Mohamed A. Elmonem Koenraad R. P. Veys Giusi Prencipe |
| author_facet | Mohamed A. Elmonem Koenraad R. P. Veys Giusi Prencipe |
| author_sort | Mohamed A. Elmonem |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T01:44:37Z |
| format | Article |
| id | doaj.art-c52251d0e6244832a004532b0895dfe1 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-10T01:44:37Z |
| publishDate | 2022-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-c52251d0e6244832a004532b0895dfe12023-11-23T13:17:26ZengMDPI AGCells2073-44092022-01-0111219010.3390/cells11020190Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New TherapiesMohamed A. Elmonem0Koenraad R. P. Veys1Giusi Prencipe2Department of Clinical and Chemical Pathology, Faculty of Medicine, Cairo University, Cairo 11628, EgyptLaboratory of Pediatric Nephrology, Department of Development & Regeneration, KU Leuven, 3000 Leuven, BelgiumLaboratory of Immuno-Rheumatology, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyThe 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.https://www.mdpi.com/2073-4409/11/2/190macrophagesinflammasomeproximal tubular cellsendocytosisautophagyapoptosis |
| spellingShingle | Mohamed A. Elmonem Koenraad R. P. Veys Giusi Prencipe Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies Cells macrophages inflammasome proximal tubular cells endocytosis autophagy apoptosis |
| title | Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies |
| title_full | Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies |
| title_fullStr | Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies |
| title_full_unstemmed | Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies |
| title_short | Nephropathic Cystinosis: Pathogenic Roles of Inflammation and Potential for New Therapies |
| title_sort | nephropathic cystinosis pathogenic roles of inflammation and potential for new therapies |
| topic | macrophages inflammasome proximal tubular cells endocytosis autophagy apoptosis |
| url | https://www.mdpi.com/2073-4409/11/2/190 |
| work_keys_str_mv | AT mohamedaelmonem nephropathiccystinosispathogenicrolesofinflammationandpotentialfornewtherapies AT koenraadrpveys nephropathiccystinosispathogenicrolesofinflammationandpotentialfornewtherapies AT giusiprencipe nephropathiccystinosispathogenicrolesofinflammationandpotentialfornewtherapies |