Minocycline Counteracts Ectopic Calcification in a Murine Model of Pseudoxanthoma Elasticum: A Proof-of-Concept Study.

ABSTRACT: Pseudoxanthoma elasticum (PXE) is an intractable Mendelian disease characterized by ectopic calcification in skin, eyes and blood vessels. Recently, increased activation of the DNA damage response (DDR) was shown to be involved in PXE pathogenesis, while the DDR/PARP1 inhibitor minocycline was found to attenuate aberrant mineralization in PXE cells and zebrafish. In this proof-of-concept study, we evaluated the anticalcifying properties of minocycline in Abcc6^-/- mice, an established mammalian PXE model. Abcc6^-/- mice received oral minocycline supplementation (40 mg/kg/day) from 12 to 36 weeks of age and were compared to untreated Abcc6^-/- and Abcc6^+/+ siblings. Ectopic calcification was evaluated using X-ray microtomo

SUBMITTER: Bouderlique E 

PROVIDER: S-EPMC8837001 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature