LAUSR

Significance In this paper, we describe a metabolic pathway that explains the long-standing puzzle as to why and how the majority of the body’s citrate is stored in mineralized tissues. The mechanism involves the coordinated actions between the membranous citrate transporter SLC13A5 and the endogenous production and export of citrate through the tricarboxylic acid cycle. Loss of function of this pathway in mice decreased bone mass and strength and disrupted the mineralization of teeth, a phenotypic feature identical to that seen in children with mutations in SLC13A5. These findings demonstrate that bone osteoblasts and tooth ameloblasts/odontoblasts function as specialized citrate-producing cells to ensure the deposition of citrate into mineral, where it is required for the proper formation of bones and teeth.