KEGG   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
HSA10329(RXYLT1)
PTR452041(RXYLT1)
PPS100992813(RXYLT1)
GGO101133488(RXYLT1)
PON100460186(RXYLT1)
NLE100584744(RXYLT1)
HMH116480811(RXYLT1)
MCC717102(RXYLT1)
MCF102139236(RXYLT1)
MTHB126930862
MNI105474028(RXYLT1)
CSAB103238633(TMEM5)
CATY105600545(TMEM5)
PANU101025195(RXYLT1)
TGE112634609(RXYLT1)
MLEU105534232(TMEM5)
RRO104680841(RXYLT1)
RBB108530040(TMEM5)
TFN117089823(RXYLT1)
PTEH111551238(RXYLT1)
CANG105502616(TMEM5)
CJC100396930(RXYLT1)
SBQ101051694(TMEM5)
CIMI108298190(RXYLT1)
CSYR103266851(TMEM5)
MMUR105880958(TMEM5)
LCAT123639566(RXYLT1)
PCOQ105824188(TMEM5)
OGA100966708(RXYLT1)
MMU216395(Rxylt1)
MCAL110303339(Rxylt1)
MPAH110326592(Tmem5)
RNO299841(Rxylt1)
MCOC116076336(Rxylt1)
ANU117696770(Rxylt1)
MUN110541232(Rxylt1) 132648666 132648675
CGE100757873(Rxylt1)
MAUA101841827(Rxylt1)
PROB127215699(Rxylt1)
PLEU114693289(Rxylt1)
MORG121455631(Rxylt1)
MFOT126494502
AAMP119803677(Rxylt1)
NGI103732350
HGL101716790(Tmem5) 106007506
CPOC100732505(Rxylt1)
CCAN109688718(Tmem5)
DORD105990711(Tmem5)
DSP122124346(Rxylt1)
PLOP125361781(Rxylt1)
NCAR124983033
MMMA107140098(Rxylt1)
OCU100345411(RXYLT1)
OPI101534296(RXYLT1)
TUP102496430(TMEM5)
GVR103608590(TMEM5)
CFA608152(RXYLT1)
CLUD112677924(RXYLT1)
VVP112922312(RXYLT1)
VLG121492180(RXYLT1)
NPO129511290(RXYLT1)
AML100464740(RXYLT1)
UMR103656829(RXYLT1)
UAH113256338(RXYLT1)
UAR123787181(RXYLT1)
ELK111159758
LLV125107638
MPUF101692107(TMEM5)
MNP132020802(RXYLT1)
MLK131838501(RXYLT1)
NVS122892001(RXYLT1)
ORO101385557(TMEM5)
EJU114214982(RXYLT1)
ZCA113923143(RXYLT1)
MLX118008632(RXYLT1)
NSU110570589(RXYLT1)
FCA101101235(RXYLT1)
PYU121040832(RXYLT1)
PCOO112863833(RXYLT1)
PBG122473937(RXYLT1)
LRUF124502540
PTG102958532(TMEM5)
PPAD109270421(TMEM5)
PUC125919283
AJU106981335(RXYLT1)
HHV120233157(RXYLT1)
BTA515147(RXYLT1)
BOM102280602(TMEM5)
BIU109559282(TMEM5)
BBUB102406645(RXYLT1)
BBIS104989805(TMEM5)
CHX102188924(TMEM5)
OAS101112124(RXYLT1)
BTAX128048643(RXYLT1)
ODA120858488(RXYLT1)
CCAD122427575(RXYLT1)
MBEZ129536622(RXYLT1)
SSC100155789(TMEM5)
CFR102515986(RXYLT1)
CBAI105081364(TMEM5)
CDK105089266(RXYLT1)
VPC102529141(RXYLT1)
BACU103019229(TMEM5)
LVE103083549(TMEM5)
OOR101289176(RXYLT1)
DLE111173799(RXYLT1)
PCAD102973659(RXYLT1) 102979593
PSIU116761140 116761329(RXYLT1)
NASI112393958(RXYLT1)
ECB100056566(RXYLT1)
EPZ103553856(TMEM5)
EAI106843751(RXYLT1)
MYB102249341(TMEM5)
MYD102767891(TMEM5)
MMYO118651451(RXYLT1)
MLF102427668(RXYLT1) 102441512
MNA107527142(TMEM5)
PKL118729942(RXYLT1)
EFUS103292562(RXYLT1)
HAI109381648(TMEM5)
DRO112319159(RXYLT1)
SHON118998717(RXYLT1)
AJM119045542(RXYLT1)
PDIC114514076(RXYLT1)
PHAS123812739(RXYLT1)
MMF118624068(RXYLT1)
RFQ117029102(RXYLT1)
PPAM129075842(RXYLT1)
PALE102877783(RXYLT1)
PGIG120605002(RXYLT1)
PVP105301072(RXYLT1)
RAY107508215(TMEM5)
MJV108403882 108408066(RXYLT1)
TOD119248114(RXYLT1)
SARA101537526(TMEM5)
LAV100665764(RXYLT1)
TMU101347097
ETF101640141(RXYLT1)
DNM101424956(RXYLT1)
GAS123248666(RXYLT1)
SHR100933243(RXYLT1)
AFZ127564284
PCW110203945 110204694 110211014(TMEM5)
OAA100086919(RXYLT1)
GGA417820(RXYLT1)
PCOC116226623(RXYLT1)
MGP100544427(RXYLT1)
CJO107310084(RXYLT1)
TPAI128073868(RXYLT1)
LMUT125685754(RXYLT1)
NMEL110392741(TMEM5)
APLA101796104(RXYLT1)
ACYG106047283(RXYLT1)
CATA118246822(RXYLT1)
AFUL116501929(RXYLT1)
TGU100218010(RXYLT1)
LSR110479854(RXYLT1)
SCAN103820429(RXYLT1)
PMOA120499876(RXYLT1)
OTC121339510(RXYLT1)
PRUF121352594(RXYLT1)
GFR102039233(TMEM5)
FAB101807811(TMEM5)
OMA130248306(RXYLT1)
PHI102110954(TMEM5)
PMAJ107205102(RXYLT1)
CCAE111932408(RXYLT1)
CCW104698351(RXYLT1)
CBRC103614876(TMEM5)
ETL114059396(RXYLT1)
ZAB102074450(RXYLT1)
ACHL103799890(TMEM5)
SVG106860663(TMEM5)
MMEA130579858(RXYLT1)
HRT120752332(RXYLT1)
FPG101921640(TMEM5)
FCH102053891(RXYLT1)
CLV102086203(TMEM5)
EGZ104135953(TMEM5)
NNI104011529(TMEM5)
PCRI104030082(TMEM5)
PLET104615153(TMEM5)
EHS104504207(TMEM5)
PCAO104041734(TMEM5)
ACUN113491375(RXYLT1)
TALA104361336(RXYLT1)
PADL103919977(TMEM5)
AFOR103900416(TMEM5)
ACHC115336229(RXYLT1)
HLE104828199(TMEM5)
AGEN126034480
GCL127019538
CCRI104159796(TMEM5)
CSTI104553898(TMEM5)
CMAC104485510(TMEM5)
MUI104538499(TMEM5)
BREG104630575(TMEM5)
FGA104083800(TMEM5)
GSTE104257914(TMEM5)
LDI104355077(TMEM5)
MNB103768433(TMEM5)
OHA104326782(TMEM5)
NNT104411615(TMEM5)
SHAB115605386(RXYLT1)
DPUB104305215(TMEM5)
PGUU104467845(TMEM5)
ACAR104529490(RXYLT1)
CPEA104388854(TMEM5)
AVIT104268716(TMEM5)
CVF104288962(TMEM5)
RTD128904082(RXYLT1)
CUCA104055181(TMEM5)
BRHI104490764(TMEM5)
AAM106488143(TMEM5)
AROW112977820(RXYLT1)
NPD112947669(RXYLT1)
DNE112978733(RXYLT1)
ASN102382915(RXYLT1)
AMJ102576823(TMEM5)
CPOO109315551(TMEM5)
GGN109295685(TMEM5)
PSS102459090(RXYLT1)
CMY102939667(RXYLT1)
CCAY125630254(RXYLT1)
DCC119843068(RXYLT1)
CPIC101945589(RXYLT1)
TST117877384(RXYLT1)
CABI116818548(RXYLT1)
MRV120384091(RXYLT1)
ACS100560359 103278900(tmem5)
ASAO132776199(RXYLT1)
PVT110075094(TMEM5)
SUND121931898(RXYLT1)
PBI103064453(RXYLT1)
PMUR107293325(RXYLT1)
CTIG120307420(RXYLT1)
TSR106543805(TMEM5)
PGUT117655858(RXYLT1)
APRI131201944(RXYLT1)
PTEX113436062(RXYLT1)
NSS113412287(RXYLT1)
VKO123019915(RXYLT1)
PMUA114605526(RXYLT1)
PRAF128422130(RXYLT1)
ZVI118091498(RXYLT1)
HCG128326052(RXYLT1)
GJA107123401(TMEM5)
STOW125435129(RXYLT1)
EMC129335968 129336015(RXYLT1)
XLA446820(rxylt1.S)
XTR100145540(rxylt1)
NPR108792582(TMEM5)
RTEM120932022(RXYLT1)
BBUF120980170(RXYLT1)
BGAR122925898(RXYLT1)
MUO115481894(RXYLT1)
GSH117347510(RXYLT1)
DRE557187(rxylt1)
SRX107722461 107738400
SANH107670366(tmem5) 107684684
SGH107559810 107587049
CCAR109110172(tmem5)
CAUA113061550 113118230
CGIB127934261 127976937
PTET122362914(rxylt1)
LROH127181247(rxylt1)
OMC131524000(rxylt1)
PPRM120462030(rxylt1)
RKG130094829(rxylt1)
MAMB125254800(rxylt1)
CIDE127499536
MASI127455910
TROS130563100(rxylt1)
TDW130435608(rxylt1)
MANU129419722(rxylt1)
IPU124627936
IFU128606557(rxylt1)
PHYP113534283(rxylt1)
SMEO124396125(rxylt1)
TFD113652270(rxylt1)
TVC132851311(rxylt1)
AMEX103039815(tmem5)
CMAO118818152(rxylt1)
EEE113577197(rxylt1)
CHAR105905127(rxylt1)
TRU101064249(rxylt1)
TFS130536196(rxylt1)
TNGGSTEN00015931G001
LCO104923719(rxylt1)
NCC104949270(tmem5)
TBEN117491339(rxylt1)
CGOB115009478(rxylt1)
PGEO117448097(rxylt1)
GACU117550792(rxylt1)
ELY117253306(rxylt1)
EFO125886990(rxylt1)
PLEP121950421(rxylt1)
SLUC116038615(rxylt1)
ECRA117948638(rxylt1)
ESP116694819(rxylt1)
PFLV114559947(rxylt1)
GAT120808735 120812786
PPUG119195733(rxylt1)
AFB129108615(rxylt1)
CLUM117732299(rxylt1)
MSAM119894254(rxylt1)
SCHU122875455(rxylt1)
CUD121514766(rxylt1)
ALAT119024414(rxylt1)
MZE101485151(rxylt1)
ONL100696362(rxylt1)
OAU116312560(rxylt1)
OLA101158942(rxylt1)
OML112152355(rxylt1)
XMA102232454(rxylt1)
XCO114155264(rxylt1)
XHE116734586(rxylt1)
PRET103466210(tmem5)
PFOR103144377(tmem5)
PLAI106956353(tmem5)
PMEI106918862(tmem5)
GAF122836255(rxylt1)
CVG107082444(tmem5)
CTUL119796790(rxylt1)
GMU124855859(rxylt1)
NFU107389076(tmem5)
KMR108237463(rxylt1)
ALIM106523189 106534997(tmem5)
NWH119415608(rxylt1)
AOCE111575928(tmem5)
MCEP125014796(rxylt1)
CSEM103380416(tmem5)
POV109641917(tmem5)
SSEN122775364(rxylt1)
HHIP117763567(rxylt1)
HSP118110149(rxylt1)
LCF108875264(rxylt1)
SDU111228597(tmem5)
SLAL111661770(rxylt1)
XGL120793739(rxylt1)
HCQ109530509(tmem5)
SSCV125971178
SBIA133501865(rxylt1)
PEE133402689(rxylt1)
PTAO133477945(rxylt1)
BPEC110161333(tmem5)
MALB109961858(tmem5)
BSPL114858005(rxylt1)
SJO128358759(rxylt1)
SASA100380601(tmem5)
STRU115152203(rxylt1) 115197402
OTW112252554(rxylt1)
OMY110523037(rxylt1)
OGO124002735(rxylt1)
ONE115108969(rxylt1)
OKI109888894(rxylt1)
OKE118401071(rxylt1)
SALP111952823(rxylt1)
SNH120054086(rxylt1)
CCLU121576979(rxylt1)
ELS105018139(rxylt1)
SFM108935981(rxylt1)
PKI111860961(rxylt1)
AANG118231063(rxylt1)
LOC102687159(tmem5)
PSPA121319998(rxylt1)
ARUT117419593(rxylt1)
PSEX120533826(rxylt1)
LCM102347491(TMEM5)
CMK103179815(rxylt1)
RTP109927331(tmem5)
CPLA122559851(rxylt1)
HOC132824625(rxylt1)
LERI129706384(rxylt1)
PMRN116949376(RXYLT1)
LRJ133342617(RXYLT1)
BFO118415550
BBEL109466277
CIN100176272
SCLV120338962
SPU583032
LPIC129255474
APLC110974336
AJC117102566
SKO100371491
BTAB109039481
CLEC106669996
HHAL106678765
NLU111058530
HVI124369162
PHUPhum_PHUM354410
FOC113211456
ZNE110831162
CSEC111867064
SGRE126297560
IEL124171814
DPXDAPPUDRAFT_48489
DMK116925727
DPZ124315743 124316312
PVM113803028
PJA122259296
PCHN125044686
PMOO119582523
HAME121854756
PCLA123762342
CQD128696150
PTRU123514028
ESN126985116
HAZT108674530
DSV119466506
RSAN119372286
RMP119163079
VDE111249696
VJA111269078
TUT107359888 107363073
DPTE113794600
DFR124490005
CSCU111635261
PTEP107455480
UDV129228128 129231499
SDM118187245
LPOL106478635
PMEO129586870
HROHELRODRAFT_191935
LGILOTGIDRAFT_228735
PVUL126826885
PCAN112571562
BGT106079271
GAE121369246
HRF124133040
HRJ124277484
CRG105323570
CVN111108246
CANU128159850 128160148
OED125654020
MYI110443872
PMAX117331769
MCAF127707624
MMER123529023
RPHI132751952
DPOL127881782
OBI106874473
OSN115216551
LAK106150449 106167194
NVE5500853 5505848
EPA110238447 110251687
ATEN116306927
ADF107327964
AMIL114956532
PDAM113679018
SPIS111323979
DGT114526485
XEN124438500 124441381
HMG101235002
TADTRIADDRAFT_19905
MBRMONBRDRAFT_16245
SREPTSG_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

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