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Mineral formation in normally non-mineralized tissues occurs when pyrophosphate cannot be produced.
The model shows arterial tissue including type I collagen and arterial smooth muscle cells. Normally, the transporter ABCC6 produces small amounts of extracellular ATP, converted to pyrophosphate and AMP by ENPP1 (right). If ENPP1 or ABCC6 are absent (left) calcium and phosphorus in the serum are adequate to cause extracellular calcification.
