Gene name: ENPP1

Uniprot entry:

P22413

Protein names:

Ectonucleotide pyrophosphatase/phosphodiesterase family member 1 (E-NPP 1) (Membrane component chromosome 6 surface marker 1) (Phosphodiesterase I/nucleotide pyrophosphatase 1) (Plasma-cell membrane glycoprotein PC-1) [Cleaved into: Ectonucleotide pyrophosphatase/phosphodiesterase family member 1, secreted form] [Includes: Alkaline phosphodiesterase I (EC 3.1.4.1); Nucleotide pyrophosphatase (NPPase) (EC 3.6.1.9) (Nucleotide diphosphatase)]

Protein sequence:

1_MERDG 6_ CAGGG 11_ SRGGE 16_ GGRAP 21_ REGPA 26_ GNGRD 31_ RGRSH 36_ AAEAP 41_ GDPQA 46_ AASLL 51_ APMDV 56_ GEEPL 61_ EKAAR 66_ ARTAK 71_ DPNTY 76_ KVLSL 81_ VLSVC 86_ VLTTI 91_ LGCIF 96_ GLKPS 101_ CAKEV 106_ KSCKG 111_ RCFER 116_ TFGNC 121_ RCDAA 126_ CVELG 131_ NCCLD 136_ YQETC 141_ IEPEH 146_ IWTCN 151_ KFRCG 156_ EKRLT 161_ RSLCA 166_ CSDDC 171_ KDKGD 176_ CCINY 181_ SSVCQ 186_ GEKSW 191_ VEEPC 196_ ESINE 201_ PQCPA 206_ GFETP 211_ PTLLF 216_ SLDGF 221_ RAEYL 226_ HTWGG 231_ LLPVI 236_ SKLKK 241_ CGTYT 246_ KNMRP 251_ VYPTK 256_ TFPNH 261_ YSIVT 266_ GLYPE 271_ SHGII 276_ DNKMY 281_ DPKMN 286_ ASFSL 291_ KSKEK 296_ FNPEW 301_ YKGEP 306_ IWVTA 311_ KYQGL 316_ KSGTF 321_ FWPGS 326_ DVEIN 331_ GIFPD 336_ IYKMY 341_ NGSVP 346_ FEERI 351_ LAVLQ 356_ WLQLP 361_ KDERP 366_ HFYTL 371_ YLEEP 376_ DSSGH 381_ SYGPV 386_ SSEVI 391_ KALQR 396_ VDGMV 401_ GMLMD 406_ GLKEL 411_ NLHRC 416_ LNLIL 421_ ISDHG 426_ MEQGS 431_ CKKYI 436_ YLNKY 441_ LGDVK 446_ NIKVI 451_ YGPAA 456_ RLRPS 461_ DVPDK 466_ YYSFN 471_ YEGIA 476_ RNLSC 481_ REPNQ 486_ HFKPY 491_ LKHFL 496_ PKRLH 501_ FAKSD 506_ RIEPL 511_ TFYLD 516_ PQWQL 521_ ALNPS 526_ ERKYC 531_ GSGFH 536_ GSDNV 541_ FSNMQ 546_ ALFVG 551_ YGPGF 556_ KHGIE 561_ ADTFE 566_ NIEVY 571_ NLMCD 576_ LLNLT 581_ PAPNN 586_ GTHGS 591_ LNHLL 596_ KNPVY 601_ TPKHP 606_ KEVHP 611_ LVQCP 616_ FTRNP 621_ RDNLG 626_ CSCNP 631_ SILPI 636_ EDFQT 641_ QFNLT 646_ VAEEK 651_ IIKHE 656_ TLPYG 661_ RPRVL 666_ QKENT 671_ ICLLS 676_ QHQFM 681_ SGYSQ 686_ DILMP 691_ LWTSY 696_ TVDRN 701_ DSFST 706_ EDFSN 711_ CLYQD 716_ FRIPL 721_ SPVHK 726_ CSFYK 731_ NNTKV 736_ SYGFL 741_ SPPQL 746_ NKNSS 751_ GIYSE 756_ ALLTT 761_ NIVPM 766_ YQSFQ 771_ VIWRY 776_ FHDTL 781_ LRKYA 786_ EERNG 791_ VNVVS 796_ GPVFD 801_ FDYDG 806_ RCDSL 811_ ENLRQ 816_ KRRVI 821_ RNQEI 826_ LIPTH 831_ FFIVL 836_ TSCKD 841_ TSQTP 846_ LHCEN 851_ LDTLA 856_ FILPH 861_ RTDNS 866_ ESCVH 871_ GKHDS 876_ SWVEE 881_ LLMLH 886_ RARIT 891_ DVEHI 896_ TGLSF 901_ YQQRK 906_ EPVSD 911_ ILKLK 916_THLPT

Protein annotations

Protein functions:

1: Nucleotide pyrophosphatase that generates diphosphate (PPi) and functions in bone mineralization and soft tissue calcification by regulating pyrophosphate levels (By similarity). PPi inhibits bone mineralization and soft tissue calcification by binding to nascent hydroxyapatite crystals, thereby preventing further growth of these crystals (PubMed:11004006). Preferentially hydrolyzes ATP, but can also hydrolyze other nucleoside 5' triphosphates such as GTP, CTP and UTP to their corresponding monophosphates with release of pyrophosphate, as well as diadenosine polyphosphates, and also 3',5'-cAMP to AMP (PubMed:25344812, PubMed:27467858, PubMed:28011303, PubMed:35147247, PubMed:8001561). 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 (PubMed:27467858, PubMed:8001561). Inhibits ectopic joint calcification and maintains articular chondrocytes by repressing hedgehog signaling; it is however unclear whether hedgehog inhibition is direct or indirect (By similarity). Appears to modulate insulin sensitivity and function (PubMed:10615944). Also involved in melanogenesis (PubMed:28964717). Also able to hydrolyze 2',3'-cGAMP (cyclic GMP-AMP), a second messenger that activates TMEM173/STING and triggers type-I interferon production (PubMed:25344812). 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 (PubMed:25344812). Not able to hydrolyze the 2',3'-cGAMP linkage isomer 3'-3'-cGAMP (PubMed:25344812)