| 靶标 | Ectonucleotide pyrophosphatase/phosphodiesterase family member 1, NPPS, PC1, NPPase, PDNP1, Membrane component chromosome 6 surface marker 1, ENPP1, Phosphodiesterase I/nucleotide pyrophosphatase 1, M6S1, Plasma-cell membrane glycoprotein PC-1, E-NPP 1, Nucleotide diphosphatase |
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| 背景 | Ectonucleotide pyrophosphatase/phosphodiesterase family member 1 (ENPP1) is a ~104 kDa protein. Nucleotide pyrophosphatase that generates diphosphate (PPi) and functions in bone mineralization and soft tissue calcification by regulating pyrophosphate levels. PPi inhibits bone mineralization and soft tissue calcification by binding to nascent hydroxyapatite crystals, thereby preventing further growth of these crystals. Preferentially hydrolyzes ATP, but can also hydrolyze other nucleoside 5' triphosphates such as GTP, CTP and UTP to their corresponding monophosphates with release of pyrophosphate, as well as diadenosine polyphosphates, and also 3',5'-cAMP to AMP. May also be involved in the regulation of the availability of nucleotide sugars in the endoplasmic reticulum and Golgi, and the regulation of purinergic signaling. Inhibits ectopic joint calcification and maintains articular chondrocytes by repressing hedgehog signaling; it is however unclear whether hedgehog inhibition is direct or indirect. 1. Johnson, KA. et al. (2000) American journal of physiology. Regulatory, integrative and comparative physiology 279, R1365-77. PMID: 110040062. Li, L. et al. (2014) Nature chemical biology 10, 1043-8. PMID: 253448123. Stella, J. et al. (2016) Human mutation 37, 1190-1201. PMID: 274678584. Namasivayam, V. et al. (2017) Biochimica et biophysica acta. General subjects 1861, 603-614. PMID: 280113035. Szeri, F. et al. (2022) Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research 37, 1024-1031. PMID: 351472476. Belli, SI. et al. (1994) European journal of biochemistry 226, 433-43. PMID: 80015618. Chourabi, M. et al. (2018) The Journal of investigative dermatology 138, 291-300. PMID: 28964717 |
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