Generation of a new Slc20a2 knockout mouse line as in vivo model for primary brain calcification.

ABSTRACT: Primary brain calcification (PBC) is a neurodegenerative disease that causes bilateral ectopic calcification in the brain. In this study, using newly generated Slc20a2 knockout (Slc20a2-/-) mice, we establish an in vivo model for PBC. In contrast to heterozygous Slc20a2+/- mice (9/9 animals) showing no obvious abnormalities, the homozygous Slc20a2-/- mice exhibited severe calcification at 11 months of age (5/5 animals). Whilst smaller in size and number, the deposits were also detectable in 5-month-old Slc20a2-/- mice (2/2 animals). By contrast, no obvious alterations were detectable in visceral organs, including the lung, kidney, liver, and spleen. Consistently, in PBC patients, despite the systemic mineral metabolic disturbance, calcification occurs only in a brain restricted manner. Hen

SUBMITTER: Kurita H 

PROVIDER: S-EPMC12369223 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature