ID A3KMS6_DANRE Unreviewed; 730 AA. AC A3KMS6; DT 03-APR-2007, integrated into UniProtKB/TrEMBL. DT 03-APR-2007, sequence version 1. DT 02-SEP-2026, entry version 89. DE SubName: Full=Exostoses (Multiple) 1a {ECO:0000313|EMBL:AAI33118.1}; GN Name=ext1a {ECO:0000313|EMBL:AAI33118.1, GN ECO:0000313|ZFIN:ZDB-GENE-050211-3}; OS Danio rerio (Zebrafish) (Brachydanio rerio). OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; OC Actinopterygii; Neopterygii; Teleostei; Ostariophysi; Cypriniformes; OC Danionidae; Danioninae; Danio. OX NCBI_TaxID=7955 {ECO:0000313|EMBL:AAI33118.1}; RN [1] {ECO:0000313|EMBL:AAI33118.1} RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. RC STRAIN=AB {ECO:0000313|EMBL:AAI33118.1}; RC TISSUE=Whole body {ECO:0000313|EMBL:AAI33118.1}; RG NIH - Zebrafish Gene Collection (ZGC) project; RL Submitted (FEB-2007) to the EMBL/GenBank/DDBJ databases. CC -!- FUNCTION: Glycosyltransferase required for the biosynthesis of heparan- CC sulfate. {ECO:0000256|ARBA:ARBA00057959}. CC -!- CATALYTIC ACTIVITY: CC Reaction=3-O-{[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n)-(1->4)- CC beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L- CC seryl-[protein] + UDP-N-acetyl-alpha-D-glucosamine = 3-O-{alpha-D- CC GlcNAc-[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n)-(1->4)-beta-D- CC GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl- CC [protein] + UDP + H(+); Xref=Rhea:RHEA:16213, Rhea:RHEA-COMP:12621, CC Rhea:RHEA-COMP:12623, ChEBI:CHEBI:15378, ChEBI:CHEBI:57705, CC ChEBI:CHEBI:58223, ChEBI:CHEBI:132415, ChEBI:CHEBI:132416; CC EC=2.4.1.224; Evidence={ECO:0000256|ARBA:ARBA00052657}; CC -!- CATALYTIC ACTIVITY: CC Reaction=3-O-{alpha-D-GlcNAc-[(1->4)-beta-D-GlcA-(1->4)-alpha-D- CC GlcNAc](n)-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal- CC (1->4)-beta-D-Xyl}-L-seryl-[protein] + UDP-alpha-D-glucuronate = 3- CC O-{[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n+1)-(1->4)-beta-D- CC GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl- CC [protein] + UDP + H(+); Xref=Rhea:RHEA:20908, Rhea:RHEA-COMP:12623, CC Rhea:RHEA-COMP:14295, ChEBI:CHEBI:15378, ChEBI:CHEBI:58052, CC ChEBI:CHEBI:58223, ChEBI:CHEBI:132415, ChEBI:CHEBI:132416; CC EC=2.4.1.225; Evidence={ECO:0000256|ARBA:ARBA00050512}; CC -!- COFACTOR: CC Name=Mn(2+); Xref=ChEBI:CHEBI:29035; CC Evidence={ECO:0000256|ARBA:ARBA00001936}; CC -!- PATHWAY: Protein modification; protein glycosylation. CC {ECO:0000256|ARBA:ARBA00004922}. CC -!- SUBCELLULAR LOCATION: Endoplasmic reticulum membrane CC {ECO:0000256|ARBA:ARBA00004648}; Single-pass type II membrane protein CC {ECO:0000256|ARBA:ARBA00004648}. CC -!- SIMILARITY: Belongs to the glycosyltransferase 47 family. CC {ECO:0000256|ARBA:ARBA00010271}. CC --------------------------------------------------------------------------- CC Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms CC Distributed under the Creative Commons Attribution (CC BY 4.0) License CC --------------------------------------------------------------------------- DR EMBL; BC133117; AAI33118.1; -; mRNA. DR RefSeq; NP_001012368.2; NM_001012368.2. DR AlphaFoldDB; A3KMS6; -. DR GeneID; 497279; -. DR KEGG; dre:497279; -. DR AGR; ZFIN:ZDB-GENE-050211-3; -. DR CTD; 497279; -. DR ZFIN; ZDB-GENE-050211-3; ext1a. DR OrthoDB; 5954868at2759; -. DR UniPathway; UPA00378; -. DR GO; GO:0005789; C:endoplasmic reticulum membrane; IEA:UniProtKB-SubCell. DR GO; GO:0005794; C:Golgi apparatus; IEA:UniProtKB-ARBA. DR GO; GO:0050508; F:glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity; IEA:UniProtKB-EC. DR GO; GO:0050509; F:N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity; IEA:UniProtKB-EC. DR GO; GO:0015012; P:heparan sulfate proteoglycan biosynthetic process; IEA:UniProtKB-ARBA. DR GO; GO:0001503; P:ossification; IEA:UniProtKB-ARBA. DR FunFam; 3.90.550.10:FF:000034; Exostosin 1; 1. DR Gene3D; 3.90.550.10; Spore Coat Polysaccharide Biosynthesis Protein SpsA, Chain A; 1. DR InterPro; IPR004263; Exostosin. DR InterPro; IPR040911; Exostosin_GT47. DR InterPro; IPR015338; GT64_dom. DR InterPro; IPR029044; Nucleotide-diphossugar_trans. DR PANTHER; PTHR48261; ACETYLGLUCOSAMINYLTRANSFERASE; 1. DR PANTHER; PTHR48261:SF3; EXOSTOSIN GLYCOSYLTRANSFERASE 1; 1. DR Pfam; PF03016; Exostosin_GT47; 1. DR Pfam; PF09258; Glyco_transf_64; 1. DR SUPFAM; SSF53448; Nucleotide-diphospho-sugar transferases; 1. PE 2: Evidence at transcript level; KW Disulfide bond {ECO:0000256|ARBA:ARBA00023157}; KW Endoplasmic reticulum {ECO:0000256|ARBA:ARBA00022824}; KW Glycoprotein {ECO:0000256|ARBA:ARBA00023180}; KW Glycosyltransferase {ECO:0000256|ARBA:ARBA00022676}; KW Manganese {ECO:0000256|ARBA:ARBA00023211}; KW Membrane {ECO:0000256|ARBA:ARBA00023136}; KW Metal-binding {ECO:0000256|ARBA:ARBA00022723}; KW Signal-anchor {ECO:0000256|ARBA:ARBA00022968}; KW Transferase {ECO:0000256|ARBA:ARBA00022679}; KW Transmembrane {ECO:0000256|ARBA:ARBA00022692}; KW Transmembrane helix {ECO:0000256|ARBA:ARBA00022989}. FT DOMAIN 95..380 FT /note="Exostosin GT47" FT /evidence="ECO:0000259|Pfam:PF03016" FT DOMAIN 464..713 FT /note="Glycosyl transferase 64" FT /evidence="ECO:0000259|Pfam:PF09258" SQ SEQUENCE 730 AA; 84591 MW; DC71F33295811657 CRC64; MQAKKRYLIL FSAGVCLILL FYLQGPASRR TPKRGDDPHP HWPHFSDPLR AFIPWDQTET EDYNVRASPR HKRDDSTGAD KCRMDSCFDF ELCKRNGFKV YVYPQQKGEK ISESYQNILS SIEGSRFYTS DPGQACLFVL NLDTLDRDQL SPQYVHNLKT KIQNLNLWNN GRNHLIFNLY SGTWPDYTED LGFDIGQAML AKASISTESF RPNFDISIPL FSKDHPRTGG ERGFLKYNTI PPFRKYMLVF KGKRYLTGIG SDTRNALYHI HNAEDVVLLT TCKHGKDWQK HKDARCDKDN AEYDRYDYKE MLHNSTFCLV PRGRRLGSFR FLEALQAACV PVMLSNGWEL PFSEIIDWRT AAVIGDERLL LQIPSTVRSI HQDRLLSLRQ QTQFLWEAYF SSVETIVLTT LEIIQDRVLQ HSAHSTLMWN RLPGGLFTLP QYSSYLGDFP FFYALLGIKP HQKFTAVIHA VTPLVSQSQP IFKLLVAVAK SQFCAQIMVL WNCDKPLPSK HRWPATSVPV IVIEGESKVM SSRFLPYENI ITDAVLSLDE DTVLSTTEVD FAFTVWQSFP ERIVGYPARS HFWDSNKERW GYTSKWTNDY SMVLTGAAFY HRYYNYLYTH YLPGSLKGLV DQLSNCEDIL MNFLVSAVTK MPPIKVTQKK QYKETMMGQT SRASRWADPD HFAQRQTCMN KFASWFGTMP LVHSQMRLDP VLFRDQVSIL RKKYRDIERL //