Ectonucleotide pyrophosphatase/phosphodiesterase 1
mammalian protein found in Homo sapiens

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.
“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.
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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.