INZ-701

INZ-701 is a recombinant fusion protein consisting of human ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1) fused to the Fc fragment of IgG1. It is being developed as an enzyme replacement therapy (ERT) for rare calcification disorders, including ENPP1 Deficiency, pseudoxanthoma elasticum (PXE), and other related conditions. The drug works by replacing deficient or dysfunctional ENPP1 enzyme activity, thereby increasing levels of inorganic pyrophosphate (PPi) and adenosine in the body. These molecules are critical regulators that inhibit pathological calcification in tissues such as blood vessels, bones, kidneys, skin, and eyes. By restoring PPi homeostasis and correcting hypophosphatemia, INZ-701 aims to prevent or reverse abnormal tissue mineralization and improve clinical outcomes in affected patients[1][2][3][4][5][6][7].

Other names

Recombinant human ectonucleotide pyrophosphatase/phosphodiesterase 1 fused to the Fc fragment of IGG1