Mouse Ectonucleotide pyrophosphatase/phosphodiesterase family member 1(ENPP1) ELISA kit

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. 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.

1. Runt-related transcription factor 2 (Runx2) was confirmed to have synergistic effects with Osx in activating ENPP1 promoter. PMID: 30047979
2. Report the temporal ectopic mineralization phenotype of connective tissues in Enpp1asj-2J mouse model for generalized arterial calcification of infancy. PMID: 27863377
3. Evidence of a causative link between ENPP1 and alterations in insulin signaling, glucose uptake, and lipid metabolism in subcutaneous abdominal Adipose tissue of gestational diabetes, which may mediate insulin resistance and hyperglycemia in Gestational diabetes. PMID: 28080219
4. Results show that urine pyrophosphate (PPI) levels are increased in Npt2a-/- mice when compared to WT, possibly to protect from renal mineralization in the setting of hyperphosphaturia. However, the presence of two hypomorphic Enpp1asj/asj alleles decreases urine PPi and worsens renal calcium phosphate deposit formation in Npt2a-/- mice. PMID: 28704395
5. Enpp1asj mutant mouse provides a new animal model for studying tympanosclerotic otitis and otitis media with effusion PMID: 27959908
6. identified ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1) as the first known mammalian enzyme lacking a NUDIX domain to generate pR from ADP-ribose on modified proteins in vitro PMID: 27406238
7. Vitamin D3 regulates Enpp1 expression, which presumably, in the context of adequate tissue non-specific alkaline phosphatase activity, provides phosphate to stimulate mineralisation. PMID: 26054750
8. ENPP1-Fc fusion protein prevents the mortality, vascular calcifications and sequela of disease in mouse models of generalized arterial calcification of infancy. PMID: 26624227
9. Expression of NPP1 and 5'-nucleotidase by valve interstitial cells promotes the mineralization of the aortic valve through A2aR and a cAMP/PKA/CREB pathway. PMID: 25644539
10. Increased NPP1 expression and activity might contribute to the decreased mineralisation observed when osteoblasts are exposed to acid conditions. PMID: 26033523
11. NPP1 has a role in obesity and diabetes in a mouse model PMID: 25368121
12. we have characterized the phenotypic and histopathologic features of this spontaneous mutant mouse, designated as asj-2J, and we have identified a large deletion/insertion mutation in the Enpp1 gene PMID: 25479107
13. these data highlight the key role of NPP1 in regulating calcification of both soft and skeletal tissues. PMID: 25260930
14. ENPP1 2'3'-cGAMP-hydrolyzing activity is repsonsible for tumor progression in humans and mice. PMID: 25344812
15. Both mono-allelic and bi-allelic ENPP1 deficiency promote dysregulated VSMC function, with robust C/EBP homologous protein expression and enhanced neointimal hyperplasia after injury in vivo, but marked post-injury calcification limited to Enpp1-/- mice. PMID: 24530784
16. Its mutaion causes osteochondral diseases and malnutrition. PMID: 24770645
17. Studied the role of genetic modulation and the role of diet in nephrocalcinosis using two established mouse models of ectopic mineralization, Abcc6(tm1Jfk) and Enpp1(asj) mice. PMID: 24732453
18. The mutant ENPP1 protein is largely absent in the liver of 'ages with stiffened joints' mice, and the lack of enzymatic activity results in reduced inorganic pyrophosphate (PPi) levels in the plasma, accompanied by extensive mineralization. PMID: 23798568
19. these data suggest a potential role for Enpp1 in the development of breast cancer bone metastasis. PMID: 23861746
20. Expression of plasma cell alloantigen 1 defines layered development of B-1a B-cell subsets with distinct innate-like functions. PMID: 23169635
21. crystal of Enpp1 belonged to space group P3(1), with unit-cell parameters a = b = 105.3, c = 173.7 A. PMID: 22750863
22. Decreased levels of nucleotide pyro- phosphatase phosphodiesterase 1 trigger osteoarthritic changes in mice. PMID: 22510396
23. NPP1 is essential for normal bone development and control of physiological bone mineralization PMID: 22359666
24. Npp1 promotes atherosclerosis, potentially mediated by Opn expression in ApoE knockout mice PMID: 21477221
25. ENPP1 plays multiple and distinct roles in the development of mineralized tissues and that the influence of ENPP1 on osteoblast differentiation and gene expression may include a mechanism that is independent of its catalytic activity. PMID: 21930712
26. RAGE promotes ectopic chondrogenic differentiation and arterial calcification in Enpp1-/- mice. PMID: 21099228
27. NPP1 does not have a major role in PP(i) generation from ATP at the level of MVs, in contrast to its accepted role on the surface of the osteoblasts and chondrocytes, but rather acts as a phosphatase in the absence of TNAP. PMID: 19874193
28. Data show that reconstitution of TNAP and NPP1 into proteoliposome membranes generates a phospholipid microenvironment that allows the kinetic study of phosphosubstrate catabolism in a manner that recapitulates the native MV microenvironment. PMID: 20048161
29. ENPP1 expression was found down-regulated during 3T3-L1 maturation, and over-expression of human ENPP1 in 3T3-L1 (pQCXIP-ENPP1 vector) resulted in adipocyte insulin resistance and in defective adipocyte maturation PMID: 17849011
30. correction of bone mineralization abnormalities in knockout mice null for both the TNAP (Akp2) and PC-1 (Enpp1) genes. PMID: 12082181
31. functional characterization of the non-catalytic ectodomains of this enzyme PMID: 12533192
32. Linked regulatory effects on extracellular PP(i) and OPN expression mediate the ability of PC-1 and ANK to regulate calcification. PMID: 12817751
33. Specific targeting motif in the NPP1 cytosolic tail that delivers PP(i)-generating NPP activity to osteoblast matrix vesicles for control of calcification. PMID: 15075217
34. NPP1 and PP(i) deficiencies modulate phenotype plasticity in artery smooth muscle cells and chondrogenesis in mesenchymal precursors PMID: 15625282
35. Lubrol resistance is an intrinsic property of NPP3 but not NPP1, which is acquired early during the biosynthetic process irrespective of its final destination, and depends on positively charged residues in its cytoplasmic tail. PMID: 17311850
36. expression of PC-1 downstream of fibroblast growth factor signaling is an integral aspect of osteoblastic function and pyrophosphate elaboration may play a significant role in the pathology of craniosynostosis PMID: 17552941
37. These results indicate that the mechanism of PC-1 induction by FGF2 in preosteoblastic cells is distinct from the mechanism of induction that occurs with osteoblast differentiation. PMID: 18698132
38. FGF2 is a specific inducer of PC-1 in pre-osteoblast cells and that FGF2 induces PC-1 expression through a mechanism involving Runx2. PMID: 19049325
39. results support a role for the Enpp1 gene in the pathogenesis associated with mineralization of articular cartilage and vascular calcification PMID: 19419305
40. Suppression of PC-1 expression improves insulin sensitivity in vitro and in an animal model of diabetes PMID: 19577557

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Defects in Enpp1 are the cause of the tiptoe walking (ttw) phenotype. Ttw mice exhibit ossification of the spinal ligaments.

[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.

Nucleotide pyrophosphatase/phosphodiesterase family

Selectively expressed on the surface of antibody-secreting cells. Expressed in osteocytes and osteoclasts.

KEGG: mmu:18605

STRING: 10090.ENSMUSP00000101159

UniGene: Mm.27254