Exostosin-1 (EC 2.4.1.224) (EC 2.4.1.225) (Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase) (Multiple exostoses protein 1) (Putative tumor suppressor protein EXT1)
1_MQAKK 6_ RYFIL 11_ LSAGS 16_ CLALL 21_ FYFGG 26_ LQFRA 31_ SRSHS 36_ RREEH 41_ SGRNG 46_ LHHPS 51_ PDHFW 56_ PRFPD 61_ ALRPF 66_ VPWDQ 71_ LENED 76_ SSVHI 81_ SPRQK 86_ RDANS 91_ SIYKG 96_ KKCRM 101_ ESCFD 106_ FTLCK 111_ KNGFK 116_ VYVYP 121_ QQKGE 126_ KIAES 131_ YQNIL 136_ AAIEG 141_ SRFYT 146_ SDPSQ 151_ ACLFV 156_ LSLDT 161_ LDRDQ 166_ LSPQY 171_ VHNLR 176_ SKVQS 181_ LHLWN 186_ NGRNH 191_ LIFNL 196_ YSGTW 201_ PDYTE 206_ DVGFD 211_ IGQAM 216_ LAKAS 221_ ISTEN 226_ FRPNF 231_ DVSIP 236_ LFSKD 241_ HPRTG 246_ GERGF 251_ LKFNT 256_ IPPLR 261_ KYMLV 266_ FKGKR 271_ YLTGI 276_ GSDTR 281_ NALYH 286_ VHNGE 291_ DVVLL 296_ TTCKH 301_ GKDWQ 306_ KHKDS 311_ RCDRD 316_ NTEYE 321_ KYDYR 326_ EMLHN 331_ ATFCL 336_ VPRGR 341_ RLGSF 346_ RFLEA 351_ LQAAC 356_ VPVML 361_ SNGWE 366_ LPFSE 371_ VINWN 376_ QAAVI 381_ GDERL 386_ LLQIP 391_ STIRS 396_ IHQDK 401_ ILALR 406_ QQTQF 411_ LWEAY 416_ FSSVE 421_ KIVLT 426_ TLEII 431_ QDRIF 436_ KHISR 441_ NSLIW 446_ NKHPG 451_ GLFVL 456_ PQYSS 461_ YLGDF 466_ PYYYA 471_ NLGLK 476_ PPSKF 481_ TAVIH 486_ AVTPL 491_ VSQSQ 496_ PVLKL 501_ LVAAA 506_ KSQYC 511_ AQIIV 516_ LWNCD 521_ KPLPA 526_ KHRWP 531_ ATAVP 536_ VVVIE 541_ GESKV 546_ MSSRF 551_ LPYDN 556_ IITDA 561_ VLSLD 566_ EDTVL 571_ STTEV 576_ DFAFT 581_ VWQSF 586_ PERIV 591_ GYPAR 596_ SHFWD 601_ NSKER 606_ WGYTS 611_ KWTND 616_ YSMVL 621_ TGAAI 626_ YHKYY 631_ HYLYS 636_ HYLPA 641_ SLKNM 646_ VDQLA 651_ NCEDI 656_ LMNFL 661_ VSAVT 666_ KLPPI 671_ KVTQK 676_ KQYKE 681_ TMMGQ 686_ TSRAS 691_ RWADP 696_ DHFAQ 701_ RQSCM 706_ NTFAS 711_ WFGYM 716_ PLIHS 721_ QMRLD 726_ PVLFK 731_ DQVSI 736_ LRKKY 741_RDIER
1: Glycosyltransferase forming with EXT2 the heterodimeric heparan sulfate polymerase which catalyzes the elongation of the heparan sulfate glycan backbone (PubMed:10639137, PubMed:22660413, PubMed:36402845, PubMed:36593275, PubMed:9620772). Glycan backbone extension consists in the alternating transfer of (1->4)-beta-D-GlcA and (1->4)-alpha-D-GlcNAc residues from their respective UDP-sugar donors. Both EXT1 and EXT2 are required for the full activity of the polymerase since EXT1 bears the N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity within the complex while EXT2 carries the glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity (PubMed:36402845, PubMed:36593275). Heparan sulfate proteoglycans are ubiquitous components of the extracellular matrix and play an important role in tissue homeostasis and signaling (PubMed:10639137, PubMed:11391482, PubMed:22660413, PubMed:9620772)