Gene Symbol: B4GAT1
Description: beta-1,4-glucuronyltransferase 1
Alias: B3GN-T1, B3GNT1, B3GNT6, BETA3GNTI, MDDGA13, iGAT, iGNT, beta-1,4-glucuronyltransferase 1, N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase, UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, beta-1,3-N-acetylglucosaminyltransferase bGnT-6, i-beta-1,3-N-acetylglucosaminyltransferase, poly-N-acetyllactosamine extension enzyme
Species: human
Products:     B4GAT1

Top Publications

  1. Sasaki K, Kurata Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, et al. Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis. Proc Natl Acad Sci U S A. 1997;94:14294-9 pubmed
    ..we describe the isolation and functional expression of a cDNA encoding beta-1,3-N-acetylglucosaminyltransferase (iGnT), an enzyme that is essential for the formation of poly-N-acetyllactosamine...
  2. Bao X, Kobayashi M, Hatakeyama S, Angata K, Gullberg D, Nakayama J, et al. Tumor suppressor function of laminin-binding alpha-dystroglycan requires a distinct beta3-N-acetylglucosaminyltransferase. Proc Natl Acad Sci U S A. 2009;106:12109-14 pubmed publisher
    ..These results identify a previously undescribed role of carbohydrate-dependent cell-BM interaction in tumor suppression and its control by beta3GnT1 and LARGE. ..
  3. Haider S, Wang J, Nagano A, Desai A, Arumugam P, Dumartin L, et al. A multi-gene signature predicts outcome in patients with pancreatic ductal adenocarcinoma. Genome Med. 2014;6:105 pubmed publisher
    ..of ITGA5, SEMA3A, KIF4A, IL20RB, SLC20A1, CDC45, PXN, SSX3 and TMEM26 was correlated with shorter survival while B3GNT1, NOSTRIN and CADPS down-regulation was associated with poor outcome...
  4. Liu X, Yu X, Xu X, Zhang X, Zhang X. The protective effects of Poria cocos-derived polysaccharide CMP33 against IBD in mice and its molecular mechanism. Food Funct. 2018;9:5936-5949 pubmed publisher
    ..Among them, 7 proteins (Hmgcs2, Fabp2, Hp, B4galnt2, B3gnt6, Sap and Ca1) were proposed to be the common targeting protein group (TPG) of CMP33 and drug SASP...
  5. Atlija M, Arranz J, Martinez Valladares M, Gutiérrez Gil B. Detection and replication of QTL underlying resistance to gastrointestinal nematodes in adult sheep using the ovine 50K SNP array. Genet Sel Evol. 2016;48:4 pubmed publisher
    ..The GWAS also revealed 10 significant SNPs associated with IgAt, although no significant associations were found for LFEC...
  6. Timbers T, Garland S, Mohan S, Flibotte S, Edgley M, Muncaster Q, et al. Accelerating Gene Discovery by Phenotyping Whole-Genome Sequenced Multi-mutation Strains and Using the Sequence Kernel Association Test (SKAT). PLoS Genet. 2016;12:e1006235 pubmed publisher
    ..Notably, BGNT-1.1 is the orthologue of human B3GNT1/B4GAT1, a glycosyltransferase associated with Walker-Warburg syndrome (WWS)...
  7. Cherian R, Jin C, Liu J, Karlsson N, Holgersson J. A Panel of Recombinant Mucins Carrying a Repertoire of Sialylated O-Glycans Based on Different Core Chains for Studies of Glycan Binding Proteins. Biomolecules. 2015;5:1810-31 pubmed publisher
    ..cells were stably transfected with plasmids encoding glycosyltransferases to synthesize core 2 (GCNT1), core 3 (B3GNT6), core 4 (GCNT1 and B3GNT6), or extended core 1 (B3GNT3) chains with or without the type 1 chain-encoding enzyme ..
  8. Nalla A, Williams T, Collins C, Rae D, Trobridge G. Lentiviral vector-mediated insertional mutagenesis screen identifies genes that influence androgen independent prostate cancer progression and predict clinical outcome. Mol Carcinog. 2016;55:1761-1771 pubmed publisher
    ..Seven out the 11 candidate genes; GLYATL1, OBSCN, STRA13, KDM2A, FAM83H, CNOT6, and B3GNT6, have not been previously implicated in PC...
  9. Lee P, Kohler J, Pfeffer S. Association of beta-1,3-N-acetylglucosaminyltransferase 1 and beta-1,4-galactosyltransferase 1, trans-Golgi enzymes involved in coupled poly-N-acetyllactosamine synthesis. Glycobiology. 2009;19:655-64 pubmed publisher
    ..residues is catalyzed by the successive action of UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1 (B3GNT1) and UDP-Gal:betaGlcNAc beta-1,4-galactosyltransferase, polypeptide 1 (B4GALT1), respectively...

More Information


  1. Buysse K, Riemersma M, Powell G, van Reeuwijk J, Chitayat D, Roscioli T, et al. Missense mutations in ?-1,3-N-acetylglucosaminyltransferase 1 (B3GNT1) cause Walker-Warburg syndrome. Hum Mol Genet. 2013;22:1746-54 pubmed publisher
    ..Here, we report two homozygous missense mutations in the ?-1,3-N-acetylglucosaminyltransferase 1 (B3GNT1) gene in a family affected with WWS. Functional studies confirmed the pathogenicity of the mutations...
  2. Praissman J, Live D, Wang S, Ramiah A, Chinoy Z, Boons G, et al. B4GAT1 is the priming enzyme for the LARGE-dependent functional glycosylation of α-dystroglycan. elife. 2014;3: pubmed publisher
    ..with catalytic activity towards β-galactose but rather a β-1,4-glucuronyltransferase, designated B4GAT1, towards both α- and β-anomers of xylose...
  3. Kammula E, Mötter J, Gorgels A, Jonas E, Hoffmann S, Willbold D. Brain transcriptome-wide screen for HIV-1 Nef protein interaction partners reveals various membrane-associated proteins. PLoS ONE. 2012;7:e51578 pubmed publisher
    ..We discuss the results with respect to many phenotypes observed in HIV infected cells and patients. The identified Nef interaction partners may help to further elucidate the molecular basis of HIV-related diseases. ..
  4. Shaheen R, Faqeih E, Ansari S, Alkuraya F. A truncating mutation in B3GNT1 causes severe Walker-Warburg syndrome. Neurogenetics. 2013;14:243-5 pubmed publisher
    ..Exome sequencing followed by autozygome filtration revealed a homozygous two-base pair insertion in B3GNT1 (NM_006876.2:c.821_822insTT), leading to premature truncation of the protein (p.Glu274Aspfs*94)...
  5. Willer T, Inamori K, Venzke D, Harvey C, Morgensen G, Hara Y, et al. The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation. elife. 2014;3: pubmed publisher
    ..Here we show that FKRP, FKTN, TMEM5 and B4GAT1 (formerly known as B3GNT1) localize to the Golgi and contribute to the O-mannosyl post-phosphorylation ..