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Ectonucleotide pyrophosphatase/phosphodiesterase 1

mammalian protein found in Homo sapiens

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Record originEnglish Wikipedia
Text licenseCC BY-SA 4.0
Source revisionSep 6, 2026
Entity authorityQ21110158 ↗
Source-derived summary

Ectonucleotide pyrophosphatase/phosphodiesterase family member 1 (PC-1, CD203a) is an enzyme that in humans is encoded by the ENPP1 gene.

Structure

This gene is a member of the ecto-nucleotide pyrophosphatase/phosphodiesterase (ENPP) family. The encoded protein is a type II transmembrane glycoprotein comprising two identical disulfide-bonded subunits.

Function

The main substrate of ENPP1 is adenosine triphosphate (ATP), which is cleaved into adenosine monophosphate (AMP) and diphosphate. Other notable substrates include cyclic guanosine monophosphate-adenosine monophosphate (cGAMP), nicotinamide adenine dinucleotide (NAD+) and cADPR.

ENPP3, a paralog of ENPP1, has also been identified as a major extracellular cGAMP hydrolase and is the second metazoan enzyme known to perform this function.

Clinical significance

Mutations in this gene have been associated with Generalized arterial calcification of infancy, ossification of the posterior longitudinal ligament of the spine (OPLL), Hypophosphatemic rickets autosomal recessive 2 (ARHR2), and insulin resistance.

In a tumor microenvironment, AMP generated by ENPP1 can lead to production of adenosine, which suppresses the anti-cancer function of the immune system.

Interactions

Ectonucleotide pyrophosphatase/phosphodiesterase 1 has been shown to interact with Insulin receptor.

Editorial summary

“Ectonucleotide pyrophosphatase/phosphodiesterase 1” enters the record as mammalian protein found in Homo sapiens. Crown Archives preserves that source wording while asking what Ectonucleotide, pyrophosphatase and phosphodiesterase can confirm, complicate or overturn.

Editorial reviewA practical starting point whose main value is the path it opens into stronger specialist and primary sources. The current 174-word lead offers orientation but no explicit four-digit date, so chronology should not be assumed. The selected authority fields contribute no independent date. Its strongest next move is a source search built around Ectonucleotide, pyrophosphatase and phosphodiesterase.
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Source & attribution

This entry incorporates text from “Ectonucleotide pyrophosphatase/phosphodiesterase 1” on English Wikipedia. Contributors are listed in the page history. Text is available under the Creative Commons Attribution-ShareAlike 4.0 License. Selected authority identifiers and statements are retrieved from Wikidata under CC0; their references and qualifiers remain part of the verification path.