DOAJ Open Access 2021

Inflammation plays a critical role in 2,8-dihydroxyadenine nephropathy

Bouderlique, Elise Tang, Ellie Perez, Joëlle Ea, Hang-Korng Renaudin, Felix +8 lainnya

Abstrak

Adenine phosphoribosyltransferase (APRT) deficiency is a genetic disease characterized by an increased production of 2,8 dihydroxyadenine (2,8-DHA) precipitating in urine, leading to a crystalline nephropathy and end-stage renal disease. Here, we describe the high prevalence of granuloma (88%) in biopsies from patients with APRT deficiency. A murine model of 2,8-DHA nephropathy was generated, showing that anakinra or dexamethasone, combined with allopurinol, improved renal function to a larger extent than allopurinol alone, the standard therapy. Inflammation plays a critical role in the development of 2,8-DHA nephropathy, and therapy based upon drugs targeting innate immunity could improve renal function recovery.

Topik & Kata Kunci

Biochemistry Physical and theoretical chemistry Mathematics

Penulis (13)

Bouderlique, Elise

Tang, Ellie

Perez, Joëlle

Ea, Hang-Korng

Renaudin, Felix

Coudert, Amélie

Vandermeersch, Sophie

Bazin, Dominique

Haymann, Jean-Philippe

Saint-Jacques, Camille

Frochot, Vincent

Daudon, Michel

Letavernier, Emmanuel

Format Sitasi

APA MLA BibTeX

Elise, B., Ellie, T., Joëlle, P., Hang-Korng, E., Felix, R., Amélie, C. et al. (2021). Inflammation plays a critical role in 2,8-dihydroxyadenine nephropathy. https://doi.org/10.5802/crchim.92

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Informasi Jurnal

Tahun Terbit: 2021
Sumber Database: DOAJ
DOI: 10.5802/crchim.92
Akses: Open Access ✓