KEGG ENZYME: 2.4.2.61

ENZYME: 2.4.2.61

Entry

EC 2.4.2.61                 Enzyme

Name

alpha-dystroglycan beta1,4-xylosyltransferase;
TMEM5 (gene name)

Class

Transferases;
Glycosyltransferases;
Pentosyltransferases

Sysname

UDP-alpha-D-xylose:3-O-[Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-O-6-P-alpha-D-Man]-Ser/Thr-[protein] xylosyltransferase

Reaction(IUBMB)

UDP-alpha-D-xylose + 3-O-[Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-O-6-P-alpha-D-Man]-Ser/Thr-[protein] = UDP + 3-O-[beta-D-Xyl-(1->4)-Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-O-6-P-alpha-D-Man]-Ser/Thr-[protein] [RN:R12244]

Reaction(KEGG)

R12244(G)

Substrate

UDP-alpha-D-xylose [CPD:C00190];
3-O-[Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-O-6-P-alpha-D-Man]-Ser/Thr-[protein] [GL:G13094]

Product

UDP [CPD:C00015];
3-O-[beta-D-Xyl-(1->4)-Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-O-6-P-alpha-D-Man]-Ser/Thr-[protein] [GL:G13095]

Comment

This eukaryotic enzyme catalyses a step in the biosynthesis of the glycan moiety of the membrane protein alpha-dystroglycan. It is specific for the second ribitol 5-phosphate in the nascent glycan chain as acceptor.

History

EC 2.4.2.61 created 2018

Pathway

ec00515

Mannose type O-glycan biosynthesis

ec01100

Metabolic pathways

Orthology

K21052

alpha-dystroglycan beta1,4-xylosyltransferase

Genes

HSA:	10329(RXYLT1)

PTR:	452041(RXYLT1)

PPS:	100992813(RXYLT1)

GGO:	101133488(RXYLT1)

PON:	100460186(RXYLT1)

NLE:	100584744(RXYLT1)

MCC:	717102(RXYLT1)

MCF:	102139236(RXYLT1)

CSAB:

103238633(TMEM5)

CATY:

105600545(TMEM5)

PANU:

101025195(RXYLT1)

TGE:	112634609(RXYLT1)

RRO:	104680841(RXYLT1)

RBB:	108530040(TMEM5)

TFN:	117089823(RXYLT1)

PTEH:

111551238(RXYLT1)

CJC:	100396930(TMEM5)

SBQ:	101051694(TMEM5)

CSYR:

103266851(TMEM5)

MMUR:

105880958(TMEM5)

OGA:	100966708(RXYLT1)

MMU:	216395(Rxylt1)

MCAL:

110303339(Tmem5)

MPAH:

110326592(Tmem5)

RNO:	299841(Rxylt1)

MCOC:

116076336(Rxylt1)

MUN:

110544358

CGE:	100757873(Rxylt1)

MAUA:

101841827(Rxylt1)

PLEU:

114693289(Rxylt1)

MORG:

121455631(Rxylt1)

NGI:

103732350

HGL:	101716790(Tmem5) 106007506

CPOC:

100732505(Rxylt1)

CCAN:

109688718(Tmem5)

DORD:

105990711(Tmem5)

DSP:	122124346(Rxylt1)

NCAR:

124983033

OCU:	100345411(TMEM5)

OPI:	101534296(RXYLT1)

TUP:	102496430(TMEM5)

CFA:	608152(RXYLT1)

VVP:	112922312(RXYLT1)

VLG:	121492180(RXYLT1)

AML:	100464740(RXYLT1)

UMR:	103656829(TMEM5)

UAH:	113256338(RXYLT1)

UAR:	123787181(RXYLT1)

ELK:

111159758

LLV:

125107638

MPUF:

101692107(TMEM5)

ORO:	101385557(TMEM5)

EJU:	114214982(RXYLT1)

ZCA:	113923143(RXYLT1)

MLX:	118008632(RXYLT1)

FCA:	101101235(TMEM5)

PYU:	121040832(RXYLT1)

PBG:	122473937(RXYLT1)

PTG:	102958532(TMEM5)

PPAD:

109270421(TMEM5)

AJU:	106981335(RXYLT1)

HHV:	120233157(RXYLT1)

BTA:	515147(RXYLT1)

BOM:	102280602(TMEM5)

BIU:	109559282(TMEM5)

BBUB:

102406645(RXYLT1)

CHX:	102188924(TMEM5)

OAS:	101112124(RXYLT1)

ODA:	120858488(RXYLT1)

CCAD:

122427575(RXYLT1)

SSC:	100155789(TMEM5)

CFR:	102515986(RXYLT1)

CBAI:

105081364(TMEM5)

CDK:	105089266(RXYLT1)

VPC:	102529141(RXYLT1)

BACU:

103019229(TMEM5)

LVE:	103083549(TMEM5)

OOR:	101289176(TMEM5)

DLE:	111173799(RXYLT1)

PCAD:

102973659(RXYLT1)

PSIU:

116761140 116761329(RXYLT1)

ECB:	100056566(RXYLT1)

EPZ:	103553856(TMEM5)

EAI:	106843751(RXYLT1)

MYB:	102249341(TMEM5)

MYD:	102767891(TMEM5)

MMYO:

118651451(RXYLT1)

MLF:	102427668(RXYLT1) 102441512

MNA:	107527142(TMEM5)

PKL:	118729942(RXYLT1)

HAI:	109381648(TMEM5)

DRO:	112319159(RXYLT1)

SHON:

118998717(RXYLT1)

AJM:	119045542(RXYLT1)

PDIC:

114514076(RXYLT1)

PHAS:

123812739(RXYLT1)

MMF:	118624068(RXYLT1)

RFQ:	117029102(RXYLT1)

PALE:

102877783(RXYLT1)

PGIG:

120605002(RXYLT1)

PVP:	105301072(RXYLT1)

RAY:	107508215(TMEM5)

MJV:	108403882 108405319(TMEM5)

TOD:	119248114(RXYLT1)

SARA:

101537526(TMEM5)

LAV:	100665764(RXYLT1)

TMU:

101347097

DNM:	101424956(RXYLT1)

GAS:	123248666(RXYLT1)

SHR:	100933243(RXYLT1)

PCW:	110203945 110204694 110211014(TMEM5)

OAA:	100086919(RXYLT1)

GGA:	417820(RXYLT1)

PCOC:

116226623(RXYLT1)

MGP:	100544427(RXYLT1)

CJO:	107310084(RXYLT1)

NMEL:

110392741(TMEM5)

APLA:

101796104(RXYLT1)

ACYG:

106047283(TMEM5)

AFUL:

116501929(RXYLT1)

TGU:	100218010(RXYLT1)

LSR:	110479854(RXYLT1)

SCAN:

103820429(RXYLT1)

PMOA:

120499876(RXYLT1)

OTC:	121339510(RXYLT1)

PRUF:

121352594(RXYLT1)

GFR:	102039233(TMEM5)

FAB:	101807811(TMEM5)

PHI:	102110954(TMEM5)

PMAJ:

107205102(TMEM5)

CCAE:

111932408(RXYLT1)

CCW:	104698351(RXYLT1)

ETL:	114059396(RXYLT1)

ZAB:	102074450(RXYLT1)

FPG:	101921640(TMEM5)

FCH:	102053891(RXYLT1)

CLV:	102086203(TMEM5)

EGZ:	104135953(TMEM5)

NNI:	104011529(TMEM5)

ACUN:

113491375(RXYLT1)

TALA:

104361336(RXYLT1)

PADL:

103919977(TMEM5)

ACHC:

115336229(RXYLT1)

AAM:	106488143(TMEM5)

AROW:

112977820(RXYLT1)

NPD:	112947669(RXYLT1)

DNE:	112978733(RXYLT1)

ASN:	102382915(RXYLT1)

AMJ:	102576823(TMEM5)

CPOO:

109315551(TMEM5)

GGN:	109295685(TMEM5)

PSS:	102459090(RXYLT1)

CMY:	102939667(RXYLT1)

CPIC:

101945589(RXYLT1)

TST:	117877384(RXYLT1)

CABI:

116818548(RXYLT1)

MRV:	120384091(RXYLT1)

ACS:	100560359 103278900(tmem5)

PVT:	110075094(TMEM5)

SUND:

121931898(RXYLT1)

PBI:	103064453(RXYLT1)

PMUR:

107293325(RXYLT1)

TSR:	106543805(TMEM5)

PGUT:

117655858(RXYLT1)

VKO:	123019915(RXYLT1)

PMUA:

114605526(RXYLT1)

ZVI:	118091498(RXYLT1)

GJA:	107123401(TMEM5)

STOW:

125435129(RXYLT1)

XLA:	446820(rxylt1.S)

XTR:	100145540(rxylt1)

NPR:	108792582(TMEM5)

RTEM:

120932022(RXYLT1)

BBUF:

120980170(RXYLT1)

BGAR:

122925898(RXYLT1)

DRE:	557187(rxylt1)

SRX:	107722461 107738400

SANH:

107670366(tmem5) 107684684

SGH:	107559810 107587049

CCAR:

109110172(tmem5)

CAUA:

113061550 113118230

PPRM:

120462030(rxylt1)

MAMB:

125254800(rxylt1)

IPU:	108264772(rxylt1) 124627936

PHYP:

113534283(rxylt1)

SMEO:

124396125(rxylt1)

TFD:	113652270(rxylt1)

AMEX:

103039815(tmem5)

EEE:	113577197(rxylt1)

TRU:	101064249(rxylt1)

TNG:	GSTEN00015931G001

LCO:	104923719(rxylt1)

NCC:	104949270(tmem5)

CGOB:

115009478(rxylt1)

ELY:	117253306(rxylt1)

PLEP:

121950421(rxylt1)

SLUC:

116038615(rxylt1)

ECRA:

117948638(rxylt1)

PFLV:

114559947(rxylt1)

GAT:	120808735 120812786

PPUG:

119195733(rxylt1)

MSAM:

119894254(rxylt1)

CUD:	121514766(rxylt1)

ALAT:

119024414(rxylt1)

MZE:	101485151(rxylt1)

ONL:	100696362(rxylt1)

OAU:	116312560(rxylt1)

OLA:	101158942(rxylt1)

OML:	112152355(rxylt1)

XMA:	102232454(rxylt1)

XCO:	114155264(rxylt1)

XHE:	116734586(rxylt1)

PRET:

103466210(tmem5)

PFOR:

103144377(tmem5)

PLAI:

106956353(tmem5)

PMEI:

106918862(tmem5)

GAF:	122836255(rxylt1)

CVG:	107082444(tmem5)

CTUL:

119796790(rxylt1)

GMU:	124855859(rxylt1)

NFU:	107389076(tmem5)

KMR:	108237463(rxylt1)

ALIM:

106523189 106534997(tmem5)

NWH:	119415608(rxylt1)

AOCE:

111575928(tmem5)

CSEM:

103380416(tmem5)

POV:	109641917(tmem5)

SSEN:

122775364(rxylt1)

HHIP:

117763567(rxylt1)

HSP:	118110149(rxylt1)

LCF:	108875264(tmem5)

SDU:	111228597(tmem5)

SLAL:

111661770(rxylt1)

XGL:	120793739(rxylt1)

HCQ:	109530509(tmem5)

BPEC:

110161333(tmem5)

MALB:

109961858(tmem5)

BSPL:

114858005(rxylt1)

SASA:

100380601(tmem5)

OTW:	112252554(rxylt1)

OMY:	110523037(rxylt1)

OGO:	124002735(rxylt1)

ONE:	115108969(rxylt1)

SALP:

111952823(rxylt1)

SNH:	120054086(rxylt1)

ELS:	105018139(rxylt1)

SFM:	108935981(rxylt1)

PKI:	111860961(rxylt1)

AANG:

118231063(rxylt1)

LOC:	102687159(tmem5)

PSPA:

121319998(rxylt1)

ARUT:

117419593(rxylt1)

LCM:	102347491(TMEM5)

CMK:	103179815(rxylt1)

RTP:	109927331(tmem5)

BFO:

118415550

BBEL:

109466277

CIN:

100176272

SCLV:

120338962

SPU:

583032

APLC:

110974336

SKO:

100371491

BTAB:

109039481

CLEC:

106669996

HHAL:

106678765

NLU:

111058530

PHU:	Phum_PHUM354410

FOC:

113211456

ZNE:

110831162

CSEC:

111867064

DPX:	DAPPUDRAFT_48489

DMK:

116925727

PVM:

113803028

PJA:

122259296

PCHN:

125044686

HAME:

121854756

PCLA:

123762342

PTRU:

123514028

HAZT:

108674530

ISC:	IscW_ISCW021970

DSV:

119466506

RSAN:

119372286

RMP:

119163079

VDE:

111249696

VJA:

111269078

TUT:	107359888 107363073

DPTE:

113794600

DFR:

124490005

CSCU:

111635261

PTEP:

107455480

SDM:

118187245

HRO:	HELRODRAFT_191935

LGI:	LOTGIDRAFT_228735

PCAN:

112571562

BGT:

106079271

GAE:

121369246

HRF:

124133040

HRJ:

124277484

CRG:

105323570

MYI:

110443872

PMAX:

117331769

MMER:

123529023

OBI:

106874473

OSN:

115216551

LAK:	106150449 106167194

NVE:	5500853 5505848

EPA:	110238447 110251687

ATEN:

116306927

ADF:

107327964

AMIL:

114956532

PDAM:

113679018

SPIS:

111323979

DGT:

114526485

XEN:	124438500 124441381

HMG:

101235002

TAD:	TRIADDRAFT_19905

MBR:	MONBRDRAFT_16245

SRE:	PTSG_08361

» show all

Reference

1 [PMID:23217329]

Authors

Vuillaumier-Barrot S, Bouchet-Seraphin C, Chelbi M, Devisme L, Quentin S, Gazal S, Laquerriere A, Fallet-Bianco C, Loget P, Odent S, Carles D, Bazin A, Aziza J, Clemenson A, Guimiot F, Bonniere M, Monnot S, Bole-Feysot C, Bernard JP, Loeuillet L, Gonzales M, Socha K, Grandchamp B, Attie-Bitach T, Encha-Razavi F, Seta N

Title

Identification of mutations in TMEM5 and ISPD as a cause of severe cobblestone lissencephaly.

Journal

Am J Hum Genet 91:1135-43 (2012)
DOI:10.1016/j.ajhg.2012.10.009

Sequence

[hsa:10329]

Reference

2 [PMID:27733679]

Authors

Manya H, Yamaguchi Y, Kanagawa M, Kobayashi K, Tajiri M, Akasaka-Manya K, Kawakami H, Mizuno M, Wada Y, Toda T, Endo T

Title

The Muscular Dystrophy Gene TMEM5 Encodes a Ribitol beta1,4-Xylosyltransferase Required for the Functional Glycosylation of Dystroglycan.

Journal

J Biol Chem 291:24618-24627 (2016)
DOI:10.1074/jbc.M116.751917

Sequence

[hsa:10329]

Other DBs

ExplorEnz - The Enzyme Database:

2.4.2.61

IUBMB Enzyme Nomenclature:

2.4.2.61

ExPASy - ENZYME nomenclature database:

2.4.2.61

BRENDA, the Enzyme Database:

2.4.2.61

All links

Pathway (5)   
   KEGG PATHWAY (4)   
   KEGG MODULE (1)   
Chemical substance (4)   
   KEGG GLYCAN (4)   
Chemical reaction (1)   
   KEGG REACTION (1)   
Gene (881)   
   KEGG ORTHOLOGY (1)   
   KEGG GENES (337)   
   KEGG MGENES (543)   
Protein sequence (17)   
   UniProt (4)   
   SWISS-PROT (3)   
   RefSeq(pep) (10)   
DNA sequence (11)   
   RefSeq(nuc) (11)   
All databases (919)   

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