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External IDsGeneCards: [1]
RefSeq (mRNA)



RefSeq (protein)



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Ectonucleotide pyrophosphatase/phosphodiesterase family member 3 is an enzyme that in humans is encoded by the ENPP3 gene.[1][2]


The protein encoded by this gene belongs to a series of ectoenzymes that are involved in hydrolysis of extracellular nucleotides. These ectoenzymes possess ATPase and ATP pyrophosphatase activities and are type II transmembrane proteins. Expression of the related rat mRNA has been found in a subset of immature glial cells and in the alimentary tract. The corresponding rat protein has been detected in the pancreas, small intestine, colon, and liver. The human mRNA is expressed in glioma cells, prostate, and uterus. Expression of the human protein has been detected in uterus, basophils, and mast cells.[2]

This protein has also been used in conjunction with CD63 as a marker for activated basophils in the Basophil Activation Test for IgE mediated allergic reactions.[3]


  1. Jin-Hua P, Goding JW, Nakamura H, Sano K (October 1997). "Molecular cloning and chromosomal localization of PD-Ibeta (PDNP3), a new member of the human phosphodiesterase I genes". Genomics. 45 (2): 412–5. doi:10.1006/geno.1997.4949. PMID 9344668.
  2. 2.0 2.1 "Entrez Gene: ENPP3 ectonucleotide pyrophosphatase/phosphodiesterase 3".
  3. McGowan EC, Saini S (February 2013). "Update on the performance and application of basophil activation tests". Current Allergy and Asthma Reports. 13 (1): 101–9. doi:10.1007/s11882-012-0324-x. PMC 4078398. PMID 23188565.

Further reading

  • Bühring HJ, Streble A, Valent P (April 2004). "The basophil-specific ectoenzyme E-NPP3 (CD203c) as a marker for cell activation and allergy diagnosis". International Archives of Allergy and Immunology. 133 (4): 317–29. doi:10.1159/000077351. PMID 15031605.
  • Andoh K, Piao JH, Terashima K, Nakamura H, Sano K (September 1999). "Genomic structure and promoter analysis of the ecto-phosphodiesterase I gene (PDNP3) expressed in glial cells". Biochimica et Biophysica Acta. 1446 (3): 213–24. doi:10.1016/s0167-4781(99)00090-1. PMID 10524196.
  • Bühring HJ, Seiffert M, Giesert C, Marxer A, Kanz L, Valent P, Sano K (May 2001). "The basophil activation marker defined by antibody 97A6 is identical to the ectonucleotide pyrophosphatase/phosphodiesterase 3". Blood. 97 (10): 3303–5. doi:10.1182/blood.V97.10.3303. PMID 11342463.
  • Yano Y, Hayashi Y, Sano K, Shinmaru H, Kuroda Y, Yokozaki H, Yoon S, Kasuga M (November 2003). "Expression and localization of ecto-nucleotide pyrophosphatase/phosphodiesterase I-3 (E-NPP3/CD203c/PD-I beta/B10/gp130RB13-6) in human colon carcinoma". International Journal of Molecular Medicine. 12 (5): 763–6. doi:10.3892/ijmm.12.5.763. PMID 14533006.
  • Yano Y, Hayashi Y, Sano K, Nagano H, Nakaji M, Seo Y, Ninomiya T, Yoon S, Yokozaki H, Kasuga M (April 2004). "Expression and localization of ecto-nucleotide pyrophosphatase/phosphodiesterase I-1 (E-NPP1/PC-1) and -3 (E-NPP3/CD203c/PD-Ibeta/B10/gp130(RB13-6)) in inflammatory and neoplastic bile duct diseases". Cancer Letters. 207 (2): 139–47. doi:10.1016/j.canlet.2003.11.002. PMID 15072822.
  • Elbatarny HS, Maurice DH (October 2005). "Leptin-mediated activation of human platelets: involvement of a leptin receptor and phosphodiesterase 3A-containing cellular signaling complex". American Journal of Physiology. Endocrinology and Metabolism. 289 (4): E695–702. doi:10.1152/ajpendo.00125.2005. PMID 15886225.
  • Kimura K, Wakamatsu A, Suzuki Y, Ota T, Nishikawa T, Yamashita R, Yamamoto J, Sekine M, Tsuritani K, Wakaguri H, Ishii S, Sugiyama T, Saito K, Isono Y, Irie R, Kushida N, Yoneyama T, Otsuka R, Kanda K, Yokoi T, Kondo H, Wagatsuma M, Murakawa K, Ishida S, Ishibashi T, Takahashi-Fujii A, Tanase T, Nagai K, Kikuchi H, Nakai K, Isogai T, Sugano S (January 2006). "Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes". Genome Research. 16 (1): 55–65. doi:10.1101/gr.4039406. PMC 1356129. PMID 16344560.
  • Ocmant A, Peignois Y, Mulier S, Hanssens L, Michils A, Schandené L (March 2007). "Flow cytometry for basophil activation markers: the measurement of CD203c up-regulation is as reliable as CD63 expression in the diagnosis of cat allergy". Journal of Immunological Methods. 320 (1–2): 40–8. doi:10.1016/j.jim.2006.12.002. PMID 17275019.