GGDB:

β3GnT1(iGnT; B3GNT1)は当初、発現クローニング法によって単離され、N-アセチルラクトサミン構造[LacNAc: Galβ1-4GlcNAc]にGlcNAcを転移し、さらに伸張することで、N-アセチルラクトサミン構造が繰り返した構造であるポリ-N-アセチルラクトサミン（ポリラクトサミン、[Galβ1-4GlcNAc]n ）を生合成するものとして報告された。しかしながら、その後、本酵素はin vitroの実験によって、b1-4結合でグルクロン酸を転移してα−ジストログリカン糖鎖の合成に関与する、B4GAT1酵素であることが報告された。B4GAT1酵素はLARGE/LARGE2の合成する２糖の繰り返し構造([GlcAb1-4Xylb1-3]n)の最初の起点となるＧｌｃＡ([GlcAb1-4Xyla1-R)の構造の合成に関与する。

Keyword: ポリ-N-アセチルラクトサミン , ポリラクトサミン

	UDP-GlcA
JCGG-STR031571 GlyTouCan: G16579HJ GlyCosmos Glycans: G16579HJ		JCGG-STR018493 GlyTouCan: G42293RF GlyCosmos Glycans: G42293RF

	UDP-GlcA
JCGG-STR033534		JCGG-STR033535

	UDP-GlcNAc
JCGG-STR013782 GlyTouCan: G00055MO GlyCosmos Glycans: G00055MO		JCGG-STR026619 GlyTouCan: G29487JS GlyCosmos Glycans: G29487JS

N-Acetylglucosaminyltransferase

GGDB Symbol

B4GAT1 Alias

Designation

UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1

Organism

Homo sapiens

GeneID

11041

HGNC

B3GNT1 (15685)

mRNA

NM_006876

map

11q13.1

Protein

NP_006867

EC#

CAZy

GT49

GlyCosmos Glycogenes

B4GAT1

OMIM

605517

Disease name

GDGDB

Human Protein Atlas

ENSG00000174684

Mus musculus

mRNA	NM_175383
Protein	NP_780592
MGI	56410

Rattus norvegicus

mRNA	XM_219693
Protein	XP_219693

Elife 1999-06-01 ;274(24):16717-26.

Poly-N-acetyllactosamine synthesis in branched N-glycans is controlled by complemental branch specificity of I-extension enzyme and beta1,4-galactosyltransferase I.

Ujita M. et al. PMID:10358011

Proc. Natl. Acad. Sci. U.S.A. 2000-05-01 ;275(21):15868-75.

Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family.

Ujita M. et al. PMID:10747980

Elife 2014-01-01 ;3():.

B4GAT1 is the priming enzyme for the LARGE-dependent functional glycosylation of α-dystroglycan.

Praissman JL. et al. PMID:25279697 Refer to: Figure 1,Figure 2

DONOR : UDP-GlcA

Glc	Man	Gal	GlcNAc	GalNAc	Fuc	Xyl	Neu5Ac	Neu5Gc	GlcA	IdoA	GlcN

Substrate Transferred

	JCGG-STR031571 GlyTouCan: G16579HJ GlyCosmos Glycans: G16579HJ	UDP-GlcA	JCGG-STR018493 GlyTouCan: G42293RF GlyCosmos Glycans: G42293RF

Proc. Natl. Acad. Sci. U.S.A. 2014-01-01 ;3():.

The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation.

Willer T. et al. PMID:25279699 Refer to: Figure 2 (B)

DONOR : UDP-GlcA

Glc	Man	Gal	GlcNAc	GalNAc	Fuc	Xyl	Neu5Ac	Neu5Gc	GlcA	IdoA	GlcN

Substrate Transferred

	JCGG-STR031571 GlyTouCan: G16579HJ GlyCosmos Glycans: G16579HJ	UDP-GlcA	JCGG-STR018493 GlyTouCan: G42293RF GlyCosmos Glycans: G42293RF

Elife 2016-03-01 ;14(9):2209-23.

Identification of a Post-translational Modification with Ribitol-Phosphate and Its Defect in Muscular Dystrophy.

Kanagawa M. et al. PMID:26923585

DONOR : UDP-GlcA

Glc	Man	Gal	GlcNAc	GalNAc	Fuc	Xyl	Neu5Ac	Neu5Gc	GlcA	IdoA	GlcN

Substrate Transferred

	JCGG-STR033534	UDP-GlcA	JCGG-STR033535

J. Biol. Chem. 1997-12-01 ;94(26):14294-9.

Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis.

Sasaki K. et al. PMID:9405606 Refer to: Fig 2

DONOR : UDP-GlcNAc

Glc	Man	Gal	GlcNAc	GalNAc	Fuc	Xyl	Neu5Ac	Neu5Gc	GlcA	IdoA	GlcN

Substrate Transferred

	JCGG-STR013782 GlyTouCan: G00055MO GlyCosmos Glycans: G00055MO	UDP-GlcNAc	JCGG-STR026619 GlyTouCan: G29487JS GlyCosmos Glycans: G29487JS

	JCGG-STR026439 GlyTouCan: G58896AZ GlyCosmos Glycans: G58896AZ	UDP-GlcNAc	JCGG-STR006200 GlyTouCan: G70874WW GlyCosmos Glycans: G70874WW

RNA Expression from Human Protein Atlas

This RNA expression data uses "HPA data" from the Human Protein Atlas. Tissue RNA Expression

data available from v22.proteinatlas.org

J. Biol. Chem. 1997-12-01 ;94(26):14294-9.

Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis.

Sasaki K. et al. PMID:9405606

braincolonfetal brainfetal kidneyfetal liverfetal lungheartkidneyliverovarypancreasplacentaprostateskeletal musclesmall intestinespleentestisthymus

Provided by GOA

Function
N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase activity
glucuronosyltransferase activity
metal ion binding

Process
axon guidance
keratan sulfate biosynthetic process
keratan sulfate synthesis
poly-N-acetyllactosamine biosynthetic process
protein O-linked mannosylation

Component
Golgi apparatus
Golgi membrane
extracellular exosome
integral component of Golgi membrane

B4GAT1

N-Acetylglucosaminyltransferase

Orthologous Gene

Mus musculus

Rattus norvegicus

Acceptor Substrates (Reference)

Poly-N-acetyllactosamine synthesis in branched N-glycans is controlled by complemental branch specificity of I-extension enzyme and beta1,4-galactosyltransferase I.

Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family.

B4GAT1 is the priming enzyme for the LARGE-dependent functional glycosylation of α-dystroglycan.

DONOR : UDP-GlcA

Substrate Transferred

The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation.

DONOR : UDP-GlcA

Substrate Transferred

Identification of a Post-translational Modification with Ribitol-Phosphate and Its Defect in Muscular Dystrophy.

DONOR : UDP-GlcA

Substrate Transferred

Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis.

DONOR : UDP-GlcNAc

Substrate Transferred

Acceptor Substrates (KEM-C)

Expression

Expression (Reference)

Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis.

Gene Ontology