GGDB:

GYLTL1B encodes glycosyltransferase-like protein LARGE2 gene, which is a paralog of LARGE gene and encodes a glycosyltransferase having β1-3-glucuronosyltransferase activity andα1,3- xylosyltransferase activity. This gene is involved in the synthesis of α−dystroglycan (phosphorylated O-mannose :N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), synthesizing (Xyla1-3GlcAb1-3)n repeats of disaccharide structure. The O-mannose on this α−dystroglycan has an important role in laminin binding, which is essential for muscle functions.

Keyword: congenital muscular dystrophy , dystroglycanopathy , phosphorylated O-mannose , α1,3-xylosyltransferase , α−dystroglycan , β1-3-glucuronosyltransferase

	UDP-Xyl
JCGG-STR031571 GlyTouCan: G16579HJ GlyCosmos Glycans: G16579HJ		JCGG-STR033547

	UDP-Xyl
JCGG-STR033547		JCGG-STR033548

	UDP-GlcA
JCGG-STR033548		JCGG-STR033549

GlucuronyltransferaseXylosyltransferase

GGDB Symbol

GYLTL1B Alias

Designation

glycosyltransferase-like 1B

Organism

Homo sapiens

GeneID

120071

HGNC

GYLTL1B (16522)

mRNA

NM_152312

map

11p11.2

Protein

NP_689525

EC#

CAZy

GlyCosmos Glycogenes

GYLTL1B

OMIM

609709

Disease name

GDGDB

Human Protein Atlas

ENSG00000165905

Mus musculus

mRNA	NM_172670
Protein	NP_766258
MGI	84827

Rattus norvegicus

mRNA	NM_199107
Protein	NP_954538

Glycobiology 2005-04-01 ;329(3):1162-71.

LARGE2 facilitates the maturation of alpha-dystroglycan more effectively than LARGE.

Fujimura K. et al. PMID:15752776

Biochem. Biophys. Res. Commun. 2012-01-01 ;335(6064):93-6.

Dystroglycan function requires xylosyl- and glucuronyltransferase activities of LARGE.

Inamori K. et al. PMID:22223806 Refer to: Figure 2

DONOR : UDP-GlcA UDP-Xyl

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

Substrate Transferred

	JCGG-STR031571 GlyTouCan: G16579HJ GlyCosmos Glycans: G16579HJ	UDP-Xyl	JCGG-STR033547

	JCGG-STR033547	UDP-GlcA	JCGG-STR033548

	JCGG-STR033548	UDP-Xyl	JCGG-STR033549

Biochem. Biophys. Res. Commun. 2013-03-01 ;23(3):295-302.

Xylosyl- and glucuronyltransferase functions of LARGE in α-dystroglycan modification are conserved in LARGE2.

Inamori K. et al. PMID:23125099

Biochem. Biophys. Res. Commun. 2013-03-01 ;23(3):303-9.

LARGE2 generates the same xylose- and glucuronic acid-containing glycan structures as LARGE.

Ashikov A. et al. PMID:23135544

Biochem. Biophys. Res. Commun. 2014-10-01 ;289(41):28138-48.

Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of α-dystroglycan in cells and tissues.

Inamori K. et al. PMID:25138275

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

Glycobiology 2005-04-01 ;329(3):1162-71.

LARGE2 facilitates the maturation of alpha-dystroglycan more effectively than LARGE.

Fujimura K. et al. PMID:15752776

adrenal glandsbone marrowfetal liverkidneylivermammary glandpancreasplacentasalivary glandskeletal musclesmall intestinespleentestisthymusthyroid glandtracheauterus

Provided by GOA

Function
UDP-xylosyltransferase activity
glucuronosyltransferase activity
manganese ion binding
xylosyltransferase activity

Process
muscle cell cellular homeostasis
protein O-linked glycosylation
protein O-linked mannosylation

Component
Golgi membrane
integral component of membrane

GYLTL1B

GlucuronyltransferaseXylosyltransferase

Orthologous Gene

Mus musculus

Rattus norvegicus

Acceptor Substrates (Reference)

LARGE2 facilitates the maturation of alpha-dystroglycan more effectively than LARGE.

Dystroglycan function requires xylosyl- and glucuronyltransferase activities of LARGE.

DONOR : UDP-GlcA UDP-Xyl

Substrate Transferred

Xylosyl- and glucuronyltransferase functions of LARGE in α-dystroglycan modification are conserved in LARGE2.

LARGE2 generates the same xylose- and glucuronic acid-containing glycan structures as LARGE.

Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of α-dystroglycan in cells and tissues.

Acceptor Substrates (KEM-C)

Expression

Expression (Reference)

LARGE2 facilitates the maturation of alpha-dystroglycan more effectively than LARGE.

Gene Ontology