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Gene Review

Enpp1  -  ectonucleotide pyrophosphatase/phosphodies...

Mus musculus

Synonyms: 4833416E15Rik, AI428932, C76301, CD203c, E-NPP 1, ...
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Disease relevance of Enpp1


High impact information on Enpp1

  • Mutation in Npps in a mouse model of ossification of the posterior longitudinal ligament of the spine [5].
  • Using a positional candidate-gene approach, we determined the ttw phenotype is caused by a nonsense mutation (glycine 568 to stop) in the Npps gene which encodes nucleotide pyrophosphatase [5].
  • Cultured Cst10-/- chondrocytes showed a reduction in calcification with activation of an SRY site in the promoter of the gene encoding nucleotide pyrophosphatase phosphodiesterase 1 (NPP1, encoded by Enpp1) [6].
  • Each allele of Akp2 and Enpp1 has a measurable influence on mineralization status in vivo [2].
  • Two osteoblast proteins, tissue-nonspecific alkaline phosphatase (TNAP) and plasma cell membrane glycoprotein-1 (PC-1) are involved in this process [2].

Chemical compound and disease context of Enpp1

  • Neuroendocrine (NE) cells may be involved not only in growth and differentiation of the normal prostate but also in carcinogenesis and progression of prostate adenocarcinoma (Pca), including development of androgen resistance [7].

Biological context of Enpp1


Anatomical context of Enpp1


Associations of Enpp1 with chemical compounds


Regulatory relationships of Enpp1

  • FGF2 specifically induced expression of PC-1 and ANK while inhibiting expression of TNAP, at both mRNA and protein levels [15].

Other interactions of Enpp1

  • The assessments of insulin, PC-1 and PC-2 mRNAs of the grafts suggest that the iNOS-/- islets may be more resistant to destruction in the transplantation model used; however, this was not sufficient to prolong the period of normoglycemia posttransplantation [16].
  • Mouse mutant ttw (tiptoe walking) is an excellent model for ectopic ossification [17].
  • Linked deficiencies in extracellular PP(i) and osteopontin mediate pathologic calcification associated with defective PC-1 and ANK expression [4].
  • Here, we report a novel allele of mouse Pc1 (N222D) that leads to obesity, abnormal proinsulin processing and multiple endocrinological defects [18].
  • It is suggested that the PC-1 protein and the transferrin receptor may have had a common evolutionary origin, and may have similar functions [14].

Analytical, diagnostic and therapeutic context of Enpp1


  1. Residues determining the binding specificity of uncompetitive inhibitors to tissue-nonspecific alkaline phosphatase. Kozlenkov, A., Le Du, M.H., Cuniasse, P., Ny, T., Hoylaerts, M.F., Millán, J.L. J. Bone Miner. Res. (2004) [Pubmed]
  2. Tissue-nonspecific alkaline phosphatase and plasma cell membrane glycoprotein-1 are central antagonistic regulators of bone mineralization. Hessle, L., Johnson, K.A., Anderson, H.C., Narisawa, S., Sali, A., Goding, J.W., Terkeltaub, R., Millan, J.L. Proc. Natl. Acad. Sci. U.S.A. (2002) [Pubmed]
  3. Calcitonin simultaneously regulates both periosteal hyperostosis and trabecular osteopenia in the spinal hyperostotic mouse (twy/twy) in vivo. Okawa, A., Goto, S., Moriya, H. Calcif. Tissue Int. (1999) [Pubmed]
  4. Linked deficiencies in extracellular PP(i) and osteopontin mediate pathologic calcification associated with defective PC-1 and ANK expression. Johnson, K., Goding, J., Van Etten, D., Sali, A., Hu, S.I., Farley, D., Krug, H., Hessle, L., Millán, J.L., Terkeltaub, R. J. Bone Miner. Res. (2003) [Pubmed]
  5. Mutation in Npps in a mouse model of ossification of the posterior longitudinal ligament of the spine. Okawa, A., Nakamura, I., Goto, S., Moriya, H., Nakamura, Y., Ikegawa, S. Nat. Genet. (1998) [Pubmed]
  6. Carminerin contributes to chondrocyte calcification during endochondral ossification. Yamada, T., Kawano, H., Koshizuka, Y., Fukuda, T., Yoshimura, K., Kamekura, S., Saito, T., Ikeda, T., Kawasaki, Y., Azuma, Y., Ikegawa, S., Hoshi, K., Chung, U.I., Nakamura, K., Kato, S., Kawaguchi, H. Nat. Med. (2006) [Pubmed]
  7. A probasin-large T antigen transgenic mouse line develops prostate adenocarcinoma and neuroendocrine carcinoma with metastatic potential. Masumori, N., Thomas, T.Z., Chaurand, P., Case, T., Paul, M., Kasper, S., Caprioli, R.M., Tsukamoto, T., Shappell, S.B., Matusik, R.J. Cancer Res. (2001) [Pubmed]
  8. Gene expression of noncollagenous bone matrix proteins in the limb joints and intervertebral disks of the twy mouse. Ohtsuki, T., Furuya, S., Yamada, T., Nomura, S., Hata, J., Yabe, Y., Hosoda, Y. Calcif. Tissue Int. (1998) [Pubmed]
  9. Chondrogenesis mediated by PPi depletion promotes spontaneous aortic calcification in NPP1-/- mice. Johnson, K., Polewski, M., van Etten, D., Terkeltaub, R. Arterioscler. Thromb. Vasc. Biol. (2005) [Pubmed]
  10. Structural and catalytic similarities between nucleotide pyrophosphatases/phosphodiesterases and alkaline phosphatases. Gijsbers, R., Ceulemans, H., Stalmans, W., Bollen, M. J. Biol. Chem. (2001) [Pubmed]
  11. Sustained osteomalacia of long bones despite major improvement in other hypophosphatasia-related mineral deficits in tissue nonspecific alkaline phosphatase/nucleotide pyrophosphatase phosphodiesterase 1 double-deficient mice. Anderson, H.C., Harmey, D., Camacho, N.P., Garimella, R., Sipe, J.B., Tague, S., Bi, X., Johnson, K., Terkeltaub, R., Millán, J.L. Am. J. Pathol. (2005) [Pubmed]
  12. Murine plasma cell membrane antigen PC-1: molecular cloning of cDNA and analysis of expression. van Driel, I.R., Wilks, A.F., Pietersz, G.A., Goding, J.W. Proc. Natl. Acad. Sci. U.S.A. (1985) [Pubmed]
  13. Identification of nucleotide pyrophosphatase/alkaline phosphodiesterase I activity associated with the mouse plasma cell differentiation antigen PC-1. Rebbe, N.F., Tong, B.D., Finley, E.M., Hickman, S. Proc. Natl. Acad. Sci. U.S.A. (1991) [Pubmed]
  14. Structure of the murine plasma cell alloantigen PC-1: comparison with the receptor for transferrin. Goding, J.W., Shen, F.W. J. Immunol. (1982) [Pubmed]
  15. FGF2 alters expression of the pyrophosphate/phosphate regulating proteins, PC-1, ANK and TNAP, in the calvarial osteoblastic cell line, MC3T3E1(C4). Hatch, N.E., Nociti, F., Swanson, E., Bothwell, M., Somerman, M. Connect. Tissue Res. (2005) [Pubmed]
  16. Survival of an islet allograft deficient in iNOS after implantation into diabetic NOD mice. Börjesson, A., Andersson, A.K., Sandler, S. Cell transplantation (2006) [Pubmed]
  17. Isolation of novel mouse genes associated with ectopic ossification by differential display method using ttw, a mouse model for ectopic ossification. Koshizuka, Y., Ikegawa, S., Sano, M., Nakamura, K., Nakamura, Y. Cytogenet. Cell Genet. (2001) [Pubmed]
  18. Obesity, hyperphagia and increased metabolic efficiency in Pc1 mutant mice. Lloyd, D.J., Bohan, S., Gekakis, N. Hum. Mol. Genet. (2006) [Pubmed]
  19. The murine plasma cell antigen PC-1: purification and partial amino acid sequence. Stearne, P.A., van Driel, I.R., Grego, B., Simpson, R.J., Goding, J.W. J. Immunol. (1985) [Pubmed]
  20. Targeted retrograde transfection of adenovirus vector carrying brain-derived neurotrophic factor gene prevents loss of mouse (twy/twy) anterior horn neurons in vivo sustaining mechanical compression. Xu, K., Uchida, K., Nakajima, H., Kobayashi, S., Baba, H. Spine (2006) [Pubmed]
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