Recombinant Human Ectonucleotide pyrophosphatase/phosphodiesterase family member 1 (ENPP1), Truncated

Uniprot Id

P22413

Target Species

Human

Target Name

ENPP1

Target Full Name

Ectonucleotide pyrophosphatase/phosphodiesterase family member 1

Target Function

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, TTP and UTP to their corresponding monophosphates with release of pyrophosphate and 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. Appears to modulate insulin sensitivity and function. Also involved in melanogenesis. Also able to hydrolyze 2'-3'-cGAMP (cyclic GMP-AMP), a second messenger that activates TMEM173/STING and triggers type-I interferon production. 2'-3'-cGAMP degradation takes place in the lumen or extracellular space, and not in the cytosol where it is produced; the role of 2'-3'-cGAMP hydrolysis is therefore unclear. Not able to hydrolyze the 2'-3'-cGAMP linkage isomer 3'-3'-cGAMP.

Target Involvement

Ossification of the posterior longitudinal ligament of the spine (OPLL); Arterial calcification of infancy, generalized, 1 (GACI1); Diabetes mellitus, non-insulin-dependent (NIDDM); Hypophosphatemic rickets, autosomal recessive, 2 (ARHR2); Cole disease (COLED)

Target Subcellular Location

[Ectonucleotide pyrophosphatase/phosphodiesterase family member 1]: Cell membrane; Single-pass type II membrane protein. Basolateral cell membrane; Single-pass type II membrane protein.; [Ectonucleotide pyrophosphatase/phosphodiesterase family member 1, secreted form]: Secreted.

Target Protein Families

Nucleotide pyrophosphatase/phosphodiesterase family

Target Tissue Specificity

Expressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis. Expressed in melanocytes but not in keratinocytes.

Target Synonyms

Alkaline phosphodiesterase 1; ARHR2; COLED; E-NPP 1; Ectonucleotide pyrophosphatase/phosphodiesterase 1; Ectonucleotide pyrophosphatase/phosphodiesterase family member 1; ENPP1; ENPP1_HUMAN; Ly 41 antigen; M6S1; Membrane component chromosome 6 surface marker 1; NPP1; NPPase; NPPS; Nucleotide pyrophosphatase; PC 1; PC-1; PCA1; PDNP1; Phosphodiesterase I/nucleotide pyrophosphatase 1; Plasma cell membrane glycoprotein 1; Plasma-cell membrane glycoprotein PC-1