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)

HMH:	116480811(RXYLT1)

MCC:	717102(RXYLT1)

MCF:	102139236(RXYLT1)

MTHB:

126930862

MNI:	105474028(RXYLT1)

CSAB:

103238633(TMEM5)

CATY:

105600545(TMEM5)

PANU:

101025195(RXYLT1)

TGE:	112634609(RXYLT1)

MLEU:

105534232(TMEM5)

RRO:	104680841(RXYLT1)

RBB:	108530040(TMEM5)

TFN:	117089823(RXYLT1)

PTEH:

111551238(RXYLT1)

CANG:

105502616(TMEM5)

CJC:	100396930(RXYLT1)

SBQ:	101051694(TMEM5)

CIMI:

108298190(RXYLT1)

CSYR:

103266851(TMEM5)

MMUR:

105880958(TMEM5)

LCAT:

123639566(RXYLT1)

PCOQ:

105824188(TMEM5)

OGA:	100966708(RXYLT1)

MMU:	216395(Rxylt1)

MCAL:

110303339(Rxylt1)

MPAH:

110326592(Tmem5)

RNO:	299841(Rxylt1)

MCOC:

116076336(Rxylt1)

ANU:	117696770(Rxylt1)

MUN:	110541232(Rxylt1) 132648666 132648675

CGE:	100757873(Rxylt1)

MAUA:

101841827(Rxylt1)

PROB:

127215699(Rxylt1)

PLEU:

114693289(Rxylt1)

MORG:

121455631(Rxylt1)

MFOT:

126494502

AAMP:

119803677(Rxylt1)

NGI:

103732350

HGL:	101716790(Tmem5) 106007506

CPOC:

100732505(Rxylt1)

CCAN:

109688718(Tmem5)

DORD:

105990711(Tmem5)

DSP:	122124346(Rxylt1)

PLOP:

125361781(Rxylt1)

NCAR:

124983033

MMMA:

107140098(Rxylt1)

OCU:	100345411(RXYLT1)

OPI:	101534296(RXYLT1)

TUP:	102496430(TMEM5)

GVR:	103608590(TMEM5)

CFA:	608152(RXYLT1)

CLUD:

112677924(RXYLT1)

VVP:	112922312(RXYLT1)

VLG:	121492180(RXYLT1)

NPO:	129511290(RXYLT1)

AML:	100464740(RXYLT1)

UMR:	103656829(RXYLT1)

UAH:	113256338(RXYLT1)

UAR:	123787181(RXYLT1)

ELK:

111159758

LLV:

125107638

MPUF:

101692107(TMEM5)

MNP:	132020802(RXYLT1)

MLK:	131838501(RXYLT1)

NVS:	122892001(RXYLT1)

ORO:	101385557(TMEM5)

EJU:	114214982(RXYLT1)

ZCA:	113923143(RXYLT1)

MLX:	118008632(RXYLT1)

NSU:	110570589(RXYLT1)

FCA:	101101235(RXYLT1)

PYU:	121040832(RXYLT1)

PCOO:

112863833(RXYLT1)

PBG:	122473937(RXYLT1)

PVIV:

125170955(RXYLT1)

LRUF:

124502540

PTG:	102958532(TMEM5)

PPAD:

109270421(TMEM5)

PUC:

125919283

AJU:	106981335(RXYLT1)

HHV:	120233157(RXYLT1)

BTA:	515147(RXYLT1)

BOM:	102280602(TMEM5)

BIU:	109559282(TMEM5)

BBUB:

102406645(RXYLT1)

BBIS:

104989805(TMEM5)

CHX:	102188924(TMEM5)

OAS:	101112124(RXYLT1)

BTAX:

128048643(RXYLT1)

ODA:	120858488(RXYLT1)

CCAD:

122427575(RXYLT1)

MBEZ:

129536622(RXYLT1)

SSC:	100155789(TMEM5)

CFR:	102515986(RXYLT1)

CBAI:

105081364(TMEM5)

CDK:	105089266(RXYLT1)

VPC:	102529141(RXYLT1)

BACU:

103019229(TMEM5)

BMUS:

118902583(RXYLT1)

LVE:	103083549(TMEM5)

OOR:	101289176(RXYLT1)

DLE:	111173799(RXYLT1)

PCAD:

102973659(RXYLT1) 102979593

PSIU:

116761140 116761329(RXYLT1)

NASI:

112393958(RXYLT1)

ECB:	100056566(RXYLT1)

EPZ:	103553856(TMEM5)

EAI:	106843751(RXYLT1)

MYB:	102249341(TMEM5)

MYD:	102767891(TMEM5)

MMYO:

118651451(RXYLT1)

MLF:	102427668(RXYLT1) 102441512

PKL:	118729942(RXYLT1)

EFUS:

103292562(RXYLT1)

MNA:	107527142(TMEM5)

DRO:	112319159(RXYLT1)

SHON:

118998717(RXYLT1)

AJM:	119045542(RXYLT1)

PDIC:

114514076(RXYLT1)

PHAS:

123812739(RXYLT1)

MMF:	118624068(RXYLT1)

PPAM:

129075842(RXYLT1)

HAI:	109381648(TMEM5)

RFQ:	117029102(RXYLT1)

PALE:

102877783(RXYLT1)

PGIG:

120605002(RXYLT1)

PVP:	105301072(RXYLT1)

RAY:	107508215(TMEM5)

MJV:	108403882 108408066(RXYLT1)

TOD:	119248114(RXYLT1)

SARA:

101537526(TMEM5)

SETR:

126021884(RXYLT1)

LAV:	100665764(RXYLT1)

TMU:

101347097

ETF:	101640141(RXYLT1)

DNM:	101424956(RXYLT1)

GAS:	123248666(RXYLT1)

SHR:	100933243(RXYLT1)

AFZ:

127564284

PCW:	110203945 110204694 110211014(TMEM5)

OAA:	100086919(RXYLT1)

GGA:	417820(RXYLT1)

PCOC:

116226623(RXYLT1)

MGP:	100544427(RXYLT1)

CJO:	107310084(RXYLT1)

TPAI:

128073868(RXYLT1)

LMUT:

125685754(RXYLT1)

NMEL:

110392741(TMEM5)

APLA:

101796104(RXYLT1)

ACYG:

106047283(RXYLT1)

CATA:

118246822(RXYLT1)

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)

OMA:	130248306(RXYLT1)

PHI:	102110954(TMEM5)

PMAJ:

107205102(RXYLT1)

CCAE:

111932408(RXYLT1)

CCW:	104698351(RXYLT1)

CBRC:

103614876(TMEM5)

ETL:	114059396(RXYLT1)

ZAB:	102074450(RXYLT1)

ACHL:

103799890(TMEM5)

SVG:	106860663(TMEM5)

MMEA:

130579858(RXYLT1)

HRT:	120752332(RXYLT1)

FPG:	101921640(TMEM5)

FCH:	102053891(RXYLT1)

CLV:	102086203(TMEM5)

EGZ:	104135953(TMEM5)

NNI:	104011529(TMEM5)

PCRI:

104030082(TMEM5)

PLET:

104615153(TMEM5)

EHS:	104504207(TMEM5)

PCAO:

104041734(TMEM5)

ACUN:

113491375(RXYLT1)

TALA:

104361336(RXYLT1)

PADL:

103919977(TMEM5)

AFOR:

103900416(TMEM5)

ACHC:

115336229(RXYLT1)

HLE:	104828199(TMEM5)

AGEN:

126034480

GCL:

127019538

CCRI:

104159796(TMEM5)

CSTI:

104553898(TMEM5)

CMAC:

104485510(TMEM5)

MUI:	104538499(TMEM5)

BREG:

104630575(TMEM5)

FGA:	104083800(TMEM5)

GSTE:

104257914(TMEM5)

LDI:	104355077(TMEM5)

MNB:	103768433(TMEM5)

OHA:	104326782(TMEM5)

NNT:	104411615(TMEM5)

SHAB:

115605386(RXYLT1)

DPUB:

104305215(TMEM5)

PGUU:

104467845(TMEM5)

ACAR:

104529490(RXYLT1)

CPEA:

104388854(TMEM5)

AVIT:

104268716(TMEM5)

CVF:	104288962(TMEM5)

RTD:	128904082(RXYLT1)

CUCA:

104055181(TMEM5)

BRHI:

104490764(TMEM5)

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)

CCAY:

125630254(RXYLT1)

DCC:	119843068(RXYLT1)

CPIC:

101945589(RXYLT1)

TST:	117877384(RXYLT1)

CABI:

116818548(RXYLT1)

MRV:	120384091(RXYLT1)

ACS:	100560359 103278900(tmem5)

ASAO:

132776199(RXYLT1)

PVT:	110075094(TMEM5)

SUND:

121931898(RXYLT1)

PBI:	103064453(RXYLT1)

PMUR:

107293325(RXYLT1)

CTIG:

120307420(RXYLT1)

TSR:	106543805(TMEM5)

PGUT:

117655858(RXYLT1)

APRI:

131201944(RXYLT1)

PTEX:

113436062(RXYLT1)

NSS:	113412287(RXYLT1)

VKO:	123019915(RXYLT1)

PMUA:

114605526(RXYLT1)

PRAF:

128422130(RXYLT1)

ZVI:	118091498(RXYLT1)

HCG:	128326052(RXYLT1)

GJA:	107123401(TMEM5)

STOW:

125435129(RXYLT1)

EMC:	129335968 129336015(RXYLT1)

XLA:	446820(rxylt1.S)

XTR:	100145540(rxylt1)

NPR:	108792582(TMEM5)

RTEM:

120932022(RXYLT1)

BBUF:

120980170(RXYLT1)

BGAR:

122925898(RXYLT1)

MUO:	115481894(RXYLT1)

GSH:	117347510(RXYLT1)

DRE:	557187(rxylt1)

SRX:	107722461 107738400

SANH:

107670366(tmem5) 107684684

SGH:	107559810 107587049

CCAR:

109110172(tmem5)

CAUA:

113061550 113118230

CGIB:

127934261 127976937

PTET:

122362914(rxylt1)

LROH:

127181247(rxylt1)

OMC:	131524000(rxylt1)

PPRM:

120462030(rxylt1)

RKG:	130094829(rxylt1)

MAMB:

125254800(rxylt1)

CIDE:

127499536

MASI:

127455910

TROS:

130563100(rxylt1)

TDW:	130435608(rxylt1)

MANU:

129419722(rxylt1)

IPU:

124627936

IFU:	128606557(rxylt1)

PHYP:

113534283(rxylt1)

SMEO:

124396125(rxylt1)

TFD:	113652270(rxylt1)

TVC:	132851311(rxylt1)

AMEX:

103039815(tmem5)

CMAO:

118818152(rxylt1)

EEE:	113577197(rxylt1)

CHAR:

105905127(rxylt1)

TRU:	101064249(rxylt1)

TFS:	130536196(rxylt1)

TNG:	GSTEN00015931G001

LCO:	104923719(rxylt1)

NCC:	104949270(tmem5)

TBEN:

117491339(rxylt1)

CGOB:

115009478(rxylt1)

PGEO:

117448097(rxylt1)

GACU:

117550792(rxylt1)

EMAC:

134881856(rxylt1)

ELY:	117253306(rxylt1)

EFO:	125886990(rxylt1)

PLEP:

121950421(rxylt1)

SLUC:

116038615(rxylt1)

ECRA:

117948638(rxylt1)

ESP:	116694819(rxylt1)

PFLV:

114559947(rxylt1)

GAT:	120808735 120812786

PPUG:

119195733(rxylt1)

AFB:	129108615(rxylt1)

CLUM:

117732299(rxylt1)

PSWI:

130195405(rxylt1)

MSAM:

119894254(rxylt1)

SCHU:

122875455(rxylt1)

CUD:	121514766(rxylt1)

ALAT:

119024414(rxylt1)

MZE:	101485151(rxylt1)

ONL:	100696362(rxylt1)

OAU:	116312560(rxylt1)

OLA:	101158942(rxylt1)

OML:	112152355(rxylt1)

CSAI:

133444679(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)

PPRL:

129364790(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)

MCEP:

125014796(rxylt1)

CSEM:

103380416(tmem5)

POV:	109641917(tmem5)

SSEN:

122775364(rxylt1)

HHIP:

117763567(rxylt1)

HSP:	118110149(rxylt1)

SMAU:

118315368(rxylt1)

LCF:	108875264(rxylt1)

SDU:	111228597(tmem5)

SLAL:

111661770(rxylt1)

XGL:	120793739(rxylt1)

HCQ:	109530509(tmem5)

SSCV:

125971178

SBIA:

133501865(rxylt1)

PEE:	133402689(rxylt1)

PTAO:

133477945(rxylt1)

BPEC:

110161333(tmem5)

MALB:

109961858(tmem5)

BSPL:

114858005(rxylt1)

SJO:	128358759(rxylt1)

SASA:

100380601(tmem5)

STRU:

115152203(rxylt1) 115197402

OTW:	112252554(rxylt1)

OMY:	110523037(rxylt1)

OGO:	124002735(rxylt1)

ONE:	115108969(rxylt1)

OKI:	109888894(rxylt1)

OKE:	118401071(rxylt1)

SALP:

111952823(rxylt1)

SNH:	120054086(rxylt1)

CCLU:

121576979(rxylt1)

ELS:	105018139(rxylt1)

SFM:	108935981(rxylt1)

PKI:	111860961(rxylt1)

AANG:

118231063(rxylt1)

LOC:	102687159(tmem5)

PSPA:

121319998(rxylt1)

ARUT:

117419593(rxylt1)

PSEX:

120533826(rxylt1)

LCM:	102347491(TMEM5)

CMK:	103179815(rxylt1)

RTP:	109927331(rxylt1)

CPLA:

122559851(rxylt1)

HOC:	132824625(rxylt1)

LERI:

129706384(rxylt1)

PMRN:

116949376(RXYLT1)

LRJ:	133342617(RXYLT1)

BFO:

118415550

BBEL:

109466277

CIN:

100176272

SCLV:

120338962

SPU:

583032

LPIC:

129255474

APLC:

110974336

AJC:

117102566

SKO:

100371491

BTAB:

109039481

CLEC:

106669996

HHAL:

106678765

NLU:

111058530

HVI:

124369162

PHU:	Phum_PHUM354410

FOC:

113211456

ZNE:

110831162

CSEC:

111867064

SGRE:

126297560

BROR:

134529340

IEL:

124171814

DPX:	DAPPUDRAFT_48489

DMK:

116925727

DPZ:	124315743 124316312

PVM:

113803028

PJA:

122259296

PCHN:

125044686

PMOO:

119582523

HAME:

121854756

PCLA:

123762342

CQD:

128696150

PTRU:

123514028

ESN:

126985116

MNZ:

135224339

HAZT:

108674530

DSV:

119466506

RSAN:

119372286

RMP:

119163079

VDE:

111249696

VJA:

111269078

TUT:	107359888 107363073

DPTE:

113794600

DFR:

124490005

CSCU:

111635261

PTEP:

107455480

ABRU:

129960783

UDV:	129228128 129231499

SDM:

118187245

LPOL:

106478635

PMEO:

129586870

HRO:	HELRODRAFT_191935

LGI:	LOTGIDRAFT_228735

PVUL:

126826885

PCAN:

112571562

BGT:

106079271

GAE:

121369246

HRF:

124133040

HRJ:

124277484

CRG:

105323570

CVN:

111108246

CANU:

128159850 128160148

OED:

125654020

MYI:

110443872

PMAX:

117331769

MCAF:

127707624

MMER:

123529023

RPHI:

132751952

DPOL:

127881782

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.