KEGG ORTHOLOGY: K01189

ORTHOLOGY: K01189

Entry

K01189                      KO

Symbol

GLA

Name

alpha-galactosidase [EC:3.2.1.22]

Pathway

map00052

Galactose metabolism

map00561

Glycerolipid metabolism

map00600

Sphingolipid metabolism

map00603

Glycosphingolipid biosynthesis - globo and isoglobo series

map01100

Metabolic pathways

map04142

Lysosome

Reaction

R01101

melibiose galactohydrolase

R01103

raffinose galactohydrolase

R01104

galactosylglycerol galactohydrolase

R01194

3-O-alpha-D-galactosyl-1D-myo-inositol galactohydrolase

R01329

epimelibiose galactohydrolase

R02926

melibiitol galactohydrolase

R03618

globotriosylceramide galactohydrolase

R03634

R04019

digalactosylceramide galactohydrolase

R04470

digalactosyl-diacylglycerol galactohydrolase

R05549

R05961

R06091

R06094

Disease

H00125

Fabry disease

H00423

Sphingolipidosis

Brite

KEGG Orthology (KO) [BR:ko00001]
09100 Metabolism
  09101 Carbohydrate metabolism
   00052 Galactose metabolism
    K01189  GLA; alpha-galactosidase
  09103 Lipid metabolism
   00561 Glycerolipid metabolism
    K01189  GLA; alpha-galactosidase
   00600 Sphingolipid metabolism
    K01189  GLA; alpha-galactosidase
  09107 Glycan biosynthesis and metabolism
   00603 Glycosphingolipid biosynthesis - globo and isoglobo series
    K01189  GLA; alpha-galactosidase
09140 Cellular Processes
  09141 Transport and catabolism
   04142 Lysosome
    K01189  GLA; alpha-galactosidase
Enzymes [BR:ko01000]
3. Hydrolases
  3.2  Glycosylases
   3.2.1  Glycosidases, i.e. enzymes that hydrolyse O- and S-glycosyl compounds
    3.2.1.22  alpha-galactosidase
     K01189  GLA; alpha-galactosidase

Other DBs

GO:

0004557

Genes

HSA:

2717(GLA)

PTR:	107971043(GLA)

PPS:	100993066(GLA)

GGO:	101141422(GLA)

PON:	100444354(GLA)

PPYG:

129024606(GLA)

NLE:	100595113(GLA)

HMH:	116811139(GLA)

SSYN:

129476406(GLA)

MCC:	703129(GLA)

MCF:	102124721(GLA)

MTHB:

126946007

MNI:	105499316(GLA)

CSAB:

103232361(GLA)

CATY:

105592033(GLA)

PANU:

101006544(GLA)

TGE:	112616156(GLA)

MLEU:

105528161(GLA)

RRO:	104659582(GLA)

RBB:	108521374(GLA)

TFN:	117085768(GLA)

PTEH:

111536493(GLA)

CANG:

105520678(GLA)

CJC:	100409841(GLA)

SBQ:	101027815(GLA)

CIMI:

108287436(GLA)

ANAN:

105707835(GLA)

CSYR:

103254976(GLA)

MMUR:

105872964(GLA)

PCOQ:

105814939(GLA)

OGA:	100960138(GLA)

MMU:	11605(Gla)

MCAL:

110286683(Gla)

MPAH:

110313589(Gla)

RNO:	363494(Gla)

MCOC:

116088640(Gla)

ANU:	117694704(Gla)

ASYL:

127674819(Gla)

MUN:	110543056(Gla)

CGE:	100752709(Gla)

MAUA:

101843049(Gla)

PROB:

127218590(Gla)

PLEU:

114697017(Gla)

MORG:

121436690 121437100

MFOT:

126493556 126503230

AAMP:

119804927(Gla)

NGI:	103746196(Gla)

HGL:	101713601(Gla)

CPOC:

100718405(Gla)

CCAN:

109686187(Gla)

DORD:

105999934(Gla)

DSP:	122096890(Gla)

DMER:

138840181(Gla)

PLOP:

125343994(Gla)

NCAR:

124972403

MMMA:

107156258(Gla)

ITI:	101967068(Gla)

OCU:	100344610 100354674

OPI:	101518562(GLA)

TUP:	102473011(GLA)

GVR:	103600399(GLA)

CLUD:

112649415(GLA)

VVP:	112925766(GLA)

VLG:	121483520(GLA)

NPO:	129518889(GLA)

AML:	100480888(GLA)

UMR:	103673000(GLA)

UAH:	113244306(GLA)

UAR:	123793931(GLA)

ELK:

111159184

LLV:

125091608

MPUF:

101693843(GLA)

MNP:	132007308(GLA)

MLK:	131821549(GLA)

NVS:	122897061(GLA)

ORO:	101367352(GLA)

EJU:	114202973 114202977

ZCA:	113930656(GLA)

MLX:	118022775(GLA)

NSU:	110585801(GLA)

LWW:	102732589(GLA)

FCA:	101091428(GLA)

PYU:	121024958(GLA)

PCOO:

112870874(GLA)

PBG:	122477649(GLA)

PVIV:

125156917(GLA)

LRUF:

124509581

LGF:	123594546(GLA)

AJU:

106984237

PTG:	102949540(GLA)

PPAD:

109258839(GLA)

PUC:

125931832

HHV:	120220881(GLA)

BTA:	532742(GLA)

BOM:	102284993(GLA)

BIU:	109555255(GLA)

BBUB:

102398241(GLA)

BBIS:

105005082(GLA)

CHX:	102175656(GLA)

OAS:	101106236(GLA)

BTAX:

128069988(GLA)

CSUM:

138070864(GLA)

ODA:	120881310(GLA)

CCAD:

122435840

MREE:

136153330(GLA)

OVR:	110124398(GLA)

MBEZ:

129564339(GLA)

SSC:	100415928(GLA)

CFR:	102523621(GLA)

CBAI:

105080779(GLA)

CDK:	105085485(GLA)

VPC:	102534614(GLA)

BACU:

103007171(GLA)

BMUS:

118889224(GLA)

LVE:	103085854(GLA)

OOR:	101282030(GLA)

LALB:

132513585(GLA)

GMF:	115843980(GLA)

DDP:	132418572(GLA)

DLE:	111178231(GLA)

PCAD:

102990477(GLA)

PSIU:

116747889(GLA)

PPHC:

136142405(GLA)

NASI:

112400673(GLA)

ECB:	100060370(GLA)

EPZ:	103542795(GLA)

EAI:	106823092(GLA)

MYB:	102248339(GLA)

MYD:	102757991(GLA)

MMYO:

118653699(GLA)

MLF:	102421834(GLA)

MDT:	132224687(GLA)

PKL:	118720039(GLA)

EFUS:

103299706(GLA)

MNA:	107523984(GLA)

DRO:	112321364(GLA)

SHON:

118996679(GLA)

AJM:	119038495(GLA)

PDIC:

114504995(GLA)

PHAS:

123805123(GLA)

MMF:	118636517(GLA)

PPAM:

129069612(GLA)

HAI:	109374543(GLA)

RFQ:	117028441(GLA)

PALE:

102878350(GLA)

PGIG:

120611558(GLA)

PVP:	105308056(GLA)

RAY:	107503618(GLA)

MJV:	108397851(GLA)

TOD:	119246026(GLA)

SARA:

101552999(GLA)

SFUM:

130032629(GLA)

SETR:

125998896(GLA)

LAV:	100654939(GLA)

TMU:

101343180

ETF:	101640086(GLA)

DNM:	101426440(GLA)

MDO:	100021325 100022779(GLA)

GAS:	123236359 123253328(GLA)

SHR:	100928928(GLA)

AFZ:

127542434

PCW:	110192314(GLA)

TVP:	118832779(GLA)

PBRV:

138143939(GLA) 138151387

MLAO:

141497588(GLA)

OAA:	100082995(GLA)

TACU:

119929610(GLA)

GGA:	422188(GLA)

PCOC:

116229563(GLA)

MGP:	100540687(GLA)

CJO:	107312562(GLA)

TPAI:

128076120(GLA)

LMUT:

125699817(GLA)

NMEL:

110403022(GLA)

APLA:

101800840(GLA)

ACYG:

106037662(GLA)

CATA:

118245634(GLA)

AFUL:

116494507(GLA)

TGU:	100218534(GLA)

LSR:	110472638(GLA)

SCAN:

103824263(GLA)

PMOA:

120506164(GLA)

OTC:	121331768(GLA)

PRUF:

121363164(GLA)

ATRC:

139597371(GLA)

GFR:	102032375(GLA)

FAB:	101807824(GLA)

OMA:	130253333(GLA)

PHI:	102110277(GLA)

PMAJ:

107203972(GLA)

CCAE:

111929403(GLA)

CCW:	104691648(GLA)

CBRC:

103617906(GLA)

ACOE:

138110547(GLA)

ETL:	114066196(GLA)

ZAB:	102067526(GLA)

ZLE:	135447909(GLA)

ACHL:

103808117(GLA)

SVG:	106853734(GLA)

MMEA:

130577307(GLA)

HRT:	120762100(GLA)

SATI:

136370515(GLA)

CCIC:

134049933(GLA)

FPG:	101911219(GLA)

FCH:	102046888(GLA)

CCRI:

104160223(GLA)

NNT:	104410959(GLA)

SHAB:

115612608(GLA)

ACUN:

113479934(GLA)

TALA:

104358210(GLA)

ACHC:

115333866(GLA)

HALD:

104322858(GLA)

HLE:	104832563(GLA)

AGEN:

126050345

HHAR:

128153853(GLA)

GCL:

127019551

LDI:	104347841(GLA)

MNB:	103770858(GLA)

DPUB:

104306355(GLA)

PPUS:

135184136(GLA)

AVIT:

104279602(GLA)

BRHI:

104489700(GLA)

EGZ:	104135421(GLA)

NNI:	104015353(GLA)

PCRI:

104024749(GLA)

PCAO:

104050826(GLA)

PADL:

103923327(GLA)

AFOR:

103904782(GLA)

FGA:	104074308(GLA)

GSTE:

104259943(GLA)

CBOY:

140658601(GLA)

CLV:	102092379(GLA)

CNIC:

135993418(GLA)

MUI:	104533739(GLA)

PGUU:

104462441(GLA)

PLET:

104617006(GLA)

CUCA:

104069018(GLA)

TEO:	104379010(GLA)

BREG:

104634021(GLA)

OHA:	104329562(GLA)

ACAR:

104531235(GLA)

CPEA:

104388336(GLA)

CVF:	104290172(GLA)

RTD:	128914723(GLA)

CRID:

134520559(GLA)

AAM:	106484973 106484974(GLA)

AROW:

112966297(GLA)

NPD:	112960428(GLA)

TGT:	104576117(GLA)

DNE:	112989700(GLA)

SCAM:

104152352(GLA)

ASN:	102383799(GLA)

AMJ:	102565208(GLA)

CPOO:

109310928(GLA)

GGN:	109300528(GLA)

PSS:	102452871(GLA)

CMY:	102945652(GLA)

CCAY:

125642262(GLA)

DCC:	119861172(GLA)

CPIC:

101931628(GLA)

TST:	117883011(GLA)

CABI:

116819984(GLA)

MRV:	120372618(GLA)

ACS:	100561589(gla)

ASAO:

132763032(GLA)

PVT:	110084915(GLA)

SUND:

121936744(GLA)

PBI:	103068265(GLA)

PMUR:

107291584(GLA)

CTIG:

120302400(GLA)

TSR:	106538895(GLA)

PGUT:

117669554(GLA)

PCAE:

139322612(GLA)

APRI:

131205154(GLA)

PTEX:

113437806(GLA)

NSS:	113416715(GLA)

VKO:	123022064(GLA)

PMUA:

114589201(GLA)

PRAF:

128406166(GLA)

ZVI:	118089020(GLA)

HCG:	128335545(GLA)

GJA:	107123930(GLA)

STOW:

125442745(GLA)

EMC:	129341269(GLA)

XLA:	734749(gla.S)

XTR:	100145767(gla)

NPR:	108805024(GLA)

RTEM:

120913078(GLA)

ACAT:

141108750

BBUF:

120977213(GLA)

BGAR:

122946395(GLA)

DTC:	141069320(GLA)

PWL:	138265624(GLA)

MUO:	115474272(GLA)

GSH:	117360984 117360986(GLA)

DRE:	450083(gla)

SRX:	107740142(gla)

SANH:

107680946(gla)

SGH:	107593238(gla)

CAUA:

113075390 113081207 113114125

CGIB:

127971961 128027729

PTET:

122357448(gla)

LROH:

127175807(gla)

GRF:	141288516(gla)

OMC:	131553423(gla)

PPRM:

120466118(gla)

RKG:	130103933(gla)

MAMB:

125259372(gla)

CIDE:

127494516

CERY:

137016985(gla)

MASI:

127412979

TROS:

130563445(gla)

TDW:	130437982(gla)

MANU:

129422468(gla)

PDAB:

135758345(gla)

CHAR:

105911386(gla)

SPIL:

134069098(gla)

TRU:	101073305(gla)

TFS:	130531552(gla)

TNG:	GSTEN00029285G001

LCO:	104919883(gla)

NCC:	104964532(gla)

TBEN:

117488530(gla)

CGOB:

115014640(gla)

PGEO:

117453774(gla)

GACU:

117541076(gla)

EMAC:

134859737(gla)

ELY:	117261455 117261479

EFO:	125894122(gla)

PLEP:

121948559(gla)

SLUC:

116052274(gla)

ECRA:

117952217(gla)

ESP:	116696733(gla)

PFLV:

114562536(gla)

GAT:	120818209(gla)

PPUG:

119211099(gla)

AFB:	129089646(gla)

CLUM:

117737144(gla)

PSWI:

130209380(gla)

CANA:

137795026(gla) 137795598

CVE:	137875676(gla)

MSAM:

119888678(gla)

SCHU:

122880467(gla)

CUD:	121516625(gla)

SPUL:

138598143(gla)

LMIX:

132982179(gla)

ALAT:

119007476(gla)

ASTR:

137603079(gla)

MZE:	101473091(gla)

ONL:	100711467(gla)

OAU:	116321029(gla)

OLA:	101169044(gla)

OML:	112153482(gla)

CSAI:

133447185(gla)

XMA:	102230586(gla)

XCO:	114139456(gla)

XHE:	116714683(gla)

PRET:

103471529(gla)

PFOR:

103151721(gla)

PLAI:

106949971(gla)

PMEI:

106920033(gla)

GAF:	122837088(gla)

PPRL:

129360714 129360723

CVG:	107093369(gla)

CTUL:

119780990(gla)

GMU:	124860751(gla)

NFU:	107377699(gla)

KMR:	108231387(gla)

ALIM:

106514532(gla)

NWH:	119408854(gla)

AOCE:

111577510(gla)

EJA:	138210965(gla)

BFRE:

138618488(gla)

MCEP:

125007891(gla)

CSEM:

103390516(gla)

POV:	109627050(gla)

SSEN:

122778797(gla)

SSOE:

131472586(gla)

HHIP:

117768791(gla)

HSP:	118112326(gla)

PPLT:

128446019(gla)

SMAU:

118319379(gla)

LCF:

108895375

SDU:	111232679(gla)

SLAL:

111648206(gla)

XGL:	120785602(gla)

HCQ:	109515848(gla)

SSCV:

125987099(gla)

SBIA:

133508503(gla)

PEE:	133408064(gla)

PTAO:

133484820(gla)

BPEC:

110158921 110159052

ENY:	140350140(gla)

MALB:

109956086(gla)

BSPL:

114864557(gla)

SJO:	128363190(gla)

SSCO:

133986010(gla)

GMH:	115552107(gla)

SASA:

100380380(gla)

STRU:

115169827(gla)

OTW:	112221096(gla)

OMY:	110488985(gla)

OGO:	123993576(gla)

ONE:	115130869(gla)

OKI:	109864894(gla)

OKE:	118380517(gla)

OMM:	135538655(gla)

OCLA:

139366407

SALP:

111965884(gla)

SNH:	120047992(gla)

CCLU:

121540726(gla)

ELS:	105025804(gla)

OEP:	134032224(gla)

SFM:	108922828(gla)

PKI:	111834969(gla)

AANG:

118223101(gla)

AROT:

135251665(gla)

CCOG:

133124112(gla)

LOC:	102689531(gla)

PSPA:

121295539 121326706

ARUT:

117430390(gla)

PSEX:

120537232(gla)

LCM:	102347864(GLA)

CPLA:

122557532(gla)

HOC:	132820185(gla)

HAKA:

140734489(gla)

PMRN:

116943621(GLA)

LRJ:

PPOI:

PVUL:

HASI:

MCAF:

RPHI:

DPOL:

Reference

PMID:3014515

Authors

Bishop DF, Calhoun DH, Bernstein HS, Hantzopoulos P, Quinn M, Desnick RJ

Title

Human alpha-galactosidase A: nucleotide sequence of a cDNA clone encoding the mature enzyme.

Journal

Proc Natl Acad Sci U S A 83:4859-63 (1986)
DOI:10.1073/pnas.83.13.4859

Sequence

[hsa:2717]

Reference

PMID:15003450

Authors

Garman SC, Garboczi DN

Title

The molecular defect leading to Fabry disease: structure of human alpha-galactosidase.

Journal

J Mol Biol 337:319-35 (2004)
DOI:10.1016/j.jmb.2004.01.035

Sequence

[hsa:2717]

All links

Ontology (1)   
   GO (1)   
Gene (838)   
   MGENES (139)   
   RefGene (694)   
   OC (5)   
All databases (839)   

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