KEGG ORTHOLOGY: K12316

ORTHOLOGY: K12316

Entry

K12316                      KO

Symbol

GAA

Name

lysosomal alpha-glucosidase [EC:3.2.1.20]

Pathway

map00052

Galactose metabolism

map00500

Starch and sucrose metabolism

map01100

Metabolic pathways

map01110

Biosynthesis of secondary metabolites

map04142

Lysosome

Disease

H00069

Glycogen storage disease

H01762

Muscle glycogen storage disease

H01940

Glycogen storage disease type II

Brite

KEGG Orthology (KO) [BR:ko00001]
09100 Metabolism
  09101 Carbohydrate metabolism
   00052 Galactose metabolism
    K12316  GAA; lysosomal alpha-glucosidase
   00500 Starch and sucrose metabolism
    K12316  GAA; lysosomal alpha-glucosidase
09140 Cellular Processes
  09141 Transport and catabolism
   04142 Lysosome
    K12316  GAA; lysosomal alpha-glucosidase
09180 Brite Hierarchies
  09183 Protein families: signaling and cellular processes
   04147 Exosome
    K12316  GAA; lysosomal alpha-glucosidase
Enzymes [BR:ko01000]
3. Hydrolases
  3.2  Glycosylases
   3.2.1  Glycosidases, i.e. enzymes that hydrolyse O- and S-glycosyl compounds
    3.2.1.20  alpha-glucosidase
     K12316  GAA; lysosomal alpha-glucosidase
Exosome [BR:ko04147]
Exosomal proteins
  Exosomal proteins of other body fluids (saliva and urine)
   K12316  GAA; lysosomal alpha-glucosidase

Other DBs

RN:	R00028 R00801 R00802

GO:

0004558

CAZy:

GH31

Genes

HSA:

2548(GAA)

PTR:	454940(GAA)

PPS:	100970564(GAA)

GGO:	101141259(GAA)

PON:	100173365(GAA)

NLE:	100584142(GAA)

MCC:	712054(GAA)

MCF:	102141245(GAA)

CSAB:

103243690(GAA)

CATY:

105588635(GAA)

PANU:

101017342(GAA)

TGE:	112609429(GAA)

RRO:	104675242(GAA)

RBB:	108534154(GAA)

TFN:	117066796(GAA)

PTEH:

111542334(GAA)

CJC:	100411176(GAA)

SBQ:	101042074(GAA)

CSYR:

103257486(GAA)

MMUR:

105866728(GAA)

OGA:	100957199(GAA)

MMU:	14387(Gaa)

MCAL:

110305854(Gaa)

MPAH:

110331633(Gaa)

RNO:	367562(Gaa)

MCOC:

116077227(Gaa)

MUN:	110550113(Gaa)

CGE:	100755105(Gaa)

MAUA:

101823184(Gaa)

PLEU:

114687821(Gaa)

MORG:

121436163(Gaa)

NGI:	103730596(Gaa)

HGL:	101718861(Gaa)

CPOC:

100728030(Gaa)

CCAN:

109684706(Gaa)

DORD:

106003122(Gaa)

DSP:	122099397(Gaa)

NCAR:

124979676

OCU:	103347590(GAA)

OPI:	101518813(GAA)

TUP:	102474055(GAA)

CFA:	483352(GAA)

VVP:	112920872(GAA)

VLG:	121473360(GAA)

AML:	100477070(GAA)

UMR:	103665978(GAA)

UAH:	113244540(GAA)

UAR:	123802990(GAA)

ELK:

111160434

LLV:

125087067

MPUF:

101689681(GAA)

ORO:	101386503(GAA)

EJU:	114216122(GAA)

ZCA:	113939457(GAA)

MLX:	118024195(GAA)

FCA:	101086359(GAA)

PYU:	121018020(GAA)

PBG:	122486176(GAA)

PTG:	102956310(GAA)

PPAD:

109276817(GAA)

AJU:	106981879(GAA)

HHV:	120228199(GAA)

BTA:	280798(GAA)

BOM:	102273281(GAA)

BIU:	109574062(GAA)

BBUB:

102407679(GAA)

CHX:	102175798(GAA)

OAS:	101121692(GAA)

ODA:	120866837(GAA)

CCAD:

122438124(GAA)

SSC:	100526132(GAA)

CFR:	102510030(GAA)

CBAI:

105065582(GAA)

CDK:	105106261(GAA)

VPC:	102525500(GAA)

BACU:

103019416(GAA)

LVE:	103090683(GAA)

OOR:	101288921(GAA)

DLE:	111182733(GAA)

PCAD:

102978552(GAA)

PSIU:

116745759(GAA)

ECB:	100056675(GAA)

EPZ:	103547327(GAA)

EAI:	106845535(GAA)

MYB:	102264084(GAA)

MYD:	102751832(GAA)

MMYO:

118672068(GAA)

MLF:	102426701(GAA)

MNA:	107532060(GAA)

PKL:	118704478(GAA)

HAI:	109373269 109383724(GAA)

DRO:	112298701(GAA)

SHON:

118999415(GAA)

AJM:	119058623(GAA)

PDIC:

114504205(GAA)

PHAS:

123817535(GAA)

MMF:	118634780(GAA)

RFQ:	117013694(GAA)

PALE:

102885829(GAA)

PGIG:

120600099(GAA)

PVP:	105305237(GAA)

RAY:	107510082(GAA)

MJV:	108387035(GAA)

TOD:	119231803(GAA)

SARA:

101544750(GAA)

LAV:	100659459(GAA)

TMU:

101344373

DNM:	101445136(GAA)

MDO:	100028995 100030818(GAA)

GAS:	123244800(GAA) 123249753

SHR:	100914209(GAA) 100933026

PCW:	110200106 110218205(GAA)

OAA:	100079324(GAA) 100083052

GGA:	416462(GAAGSD) 422082(GAA)

PCOC:

116232525(GAA) 116237588

MGP:	100547897 100548976(GAA)

CJO:	107320667 107321973(GAA)

NMEL:

110406141 110407405(GAA)

APLA:

113845291

ACYG:

106033539 106046740(GAA)

AFUL:

116495205 116496702(GAA)

TGU:	100226089 100227842(GAA)

LSR:	110472472(GAA) 110475464

SCAN:

103817641 103819935(GAA)

PMOA:

120505461(GAA) 120513087

OTC:	121334628 121338449(GAA)

PRUF:

121350845 121363413(GAA)

GFR:	102037015(GAA) 102044562

FAB:	101807850(GAA) 101820296

PHI:	102101960 102102156(GAA)

PMAJ:

107211160 107212383(GAA)

CCAE:

111936077 111944974

CCW:	104684825 104698238(GAA)

ETL:	114054771 114068226(GAA)

ZAB:	102062704 102073734(GAA)

FPG:	101918651(GAA) 101924021

FCH:	102046260 102051071(GAA)

CLV:	102090362(GAA) 102090839

EGZ:	104128958 104130629(GAA)

NNI:	104014328(GAA)

ACUN:

113485633

TALA:

104365775 116960950(GAA)

PADL:

103913521

ACHC:

115335647 115341252(GAA)

AAM:	106491310 106500263

AROW:

112973744(GAA) 112974251

NPD:	112945287(GAA) 112960030

DNE:	112990902(GAA) 112992850

ASN:	102379461 102381953(GAA)

AMJ:	102572123 102577078(GAA)

CPOO:

109314734 109315191(GAA)

GGN:	109294525 109296246(GAA)

PSS:	102459990(GAA)

CMY:	102934350 102939927(GAA)

CPIC:

101932947 101945048(GAA)

TST:	117883591 117887707(GAA)

CABI:

116832931 116834840(GAA)

MRV:	120373231 120383165(GAA)

ACS:	100553663 100561813(gaa)

PVT:	110084704 110090137(GAA)

SUND:

121914361 121922372(GAA)

PBI:	103061161 103065099(GAA)

PMUR:

107288787(GAA) 107290641

TSR:	106544831 106553676(GAA)

PGUT:

117663002(GAA) 117679547

VKO:	123025221 123036907(GAA)

PMUA:

114583983 114589000(GAA)

ZVI:	118079704(GAA) 118092842

GJA:	107118778 107119854(GAA)

STOW:

125430107(GAA) 125430994

XLA:

108703274

XTR:	100495955(gaa)

NPR:	108792966 108792971

RTEM:

120919155 120919333

BBUF:

121003349 121003350

BGAR:

122942621 122942622

DRE:	100002366(gaa) 100332784(si:ch73-12o23.1)

SRX:	107722961 107725392 107758119(gaa)

SANH:

107689991(gaa) 107692649 107702495

SGH:	107559225 107565173 107590719

CCAR:

109055938 109077732

CAUA:

113044520 113046664(gaa) 113094728

PPRM:

120463387(si:ch73-12o23.1) 120489752(gaa)

MAMB:

125264198(gaa) 125272732(gaa2)

IPU:	108258846(gaa2) 108260916(gaa)

PHYP:

113528512(gaa) 113535694

SMEO:

124377868(gaa2) 124396615(gaa)

TFD:	113639523(gaa) 113651735(gaa2)

AMEX:

103033787 103044115(gaa)

EEE:	113573276(gaa) 113574921

TRU:	101074630 101076752 105419329

TNG:	GSTEN00015697G001 GSTEN00033404G001

LCO:	104918857(gaa) 104926848

NCC:	104949822 104964793(gaa)

CGOB:

115012592 115025643

ELY:	117255627(si:ch73-12o23.1) 117269051(gaa)

PLEP:

121942064(gaa2) 121957091(gaa)

SLUC:

116042926(si:ch73-12o23.1) 116059483(gaa)

ECRA:

117958564(gaa) 117959913(si:ch73-12o23.1)

PFLV:

114546129 114569367(gaa)

GAT:	120825631(gaa2) 120827845(gaa)

PPUG:

119217879(si:ch73-12o23.1) 119220384(gaa)

MSAM:

119889149(gaa) 119896351 119911252

CUD:	121508134(gaa2) 121529629(gaa)

ALAT:

119013770(gaa) 119016995(si:ch73-12o23.1)

MZE:	101467306(gaa) 101478649

ONL:	100691577(gaa) 100695517

OAU:	116313034(gaa) 116327580(si:ch73-12o23.1)

OLA:	101161640(gaa) 101169174

OML:	112137091(si:ch73-12o23.1) 112146048(gaa)

XMA:	102223585 102231710(gaa)

XCO:	114144344 114160012(gaa)

XHE:	116719702 116735558(gaa)

PRET:

103469228(gaa) 103481270

PFOR:

103136447 103141631(gaa)

PLAI:

106957163(gaa) 106964766

PMEI:

106918766 106928874

GAF:	122822509(gaa) 122830355(gaa2)

CVG:	107092809(gaa) 107097057

CTUL:

119784684(si:ch73-12o23.1) 119798318(gaa)

GMU:	124862976(gaa) 124863726 124868660(gaa2)

NFU:	107376530(gaa) 107389057

KMR:	108231909(si:ch73-12o23.1) 108239235(gaa)

ALIM:

106518748(gaa) 106522707

NWH:	119420974(gaa) 119422961(gaa2)

AOCE:

111577359(gaa) 111588140

CSEM:

103383270 103393066(gaa)

POV:	109638725(gaa) 109642103

SSEN:

122760668 122760676 122760680 122771539(gaa2) 122785018

HHIP:

117760819(si:ch73-12o23.1) 117766593(gaa)

HSP:	118123894(gaa) 118124416(gaa2)

LCF:	108879632 108887206 108894833

SDU:	111216602 111227137(gaa)

SLAL:

111655551 111667512(gaa)

XGL:	120788392(gaa) 120800413(gaa2)

HCQ:	109511032 109522721(gaa)

BPEC:

110157747 110166448(gaa)

MALB:

109952354 109969497(gaa)

BSPL:

114856399(gaa2) 114860462(gaa)

SASA:

106564132 106588841 106595381 106601771

OTW:	112253207(gaa2) 112259065(gaa)

OMY:	110494869 110515478 110539035 118938328

OGO:	124003753 124025282

ONE:

115116423

SALP:

112073863

SNH:	120039550 120041269(gaa)

ELS:	105013030(gaa) 105029840

SFM:	108926133 108936742(gaa)

PKI:	111843280(gaa) 111845470

AANG:

118216332(gaa) 118221225(si:ch73-12o23.1)

LOC:	102697245 107078548(gaa)

PSPA:

121295973(gaa) 121300778(gaa2)

ARUT:

117424254(gaa) 117431603(si:ch73-12o23.1)

LCM:	102355272(GAA) 102366792

CMK:	103175511(gaa) 103186597(gaa2)

RTP:	109926703 109931212 109931213

BFO:	118427978 118427981 118429186

BBEL:

109476621

CIN:

100187282

SCLV:

120329290

SPU:	582502 764550

APLC:

110986134 110986153 110986154

SKO:	100366661 100371743 100372586 102804585

AGA:	AgaP_AGAP001534

ACOZ:

AARA:

ASTE:

AAG:

AALB:

CPII:

AME:

ACER:

ALAB:

BIM:

BBIF:

BVK:

BVAN:

BTER:

BPYO:

CCAL:

OBB:

MGEN:

NMEA:

CGIG:

SOC:

MPHA:

AEC:

ACEP:

PBAR:

VEM:

HST:

DQU:

CFO:

FEX:

LHU:

PGC:

OBO:

PCF:

PFUC:

VPS:

NVI:	100121715 100124137

CSOL:

TPRE:

MDL:

CGLO:

FAS:

DAM:	107042227 107044203

AGIF:

122854632

CCIN:

107264133 107273209

TCA:	657390 658192

DPA:	109537673 109539233

SOY:	115889633 115889651

ATD:

109598697

CSET:

123312765 123313525 123315869 123318673

AGB:	108907493 108911591

LDC:	111503482 111513869

NVL:	108561339 108567530

APLN:

108737535

PPYR:

116174171

OTU:	111415030 111419039

BMOR:

101736905 101744138

BMAN:

114243285 114248263

MSEX:

115447898 115454837 119188527 119189430

DPL:	KGM_204829 KGM_212394

BANY:

112046377 112049706

PMAC:

106718904

PRAP:

110995043 111003643

ZCE:	119828237 119839797

HAW:	110373713 110384254

TNL:	113497839 113503307

SLIU:

111355132

PXY:

105385777

API:	100158725 100160797

DNX:	107169248 107169249

AGS:	114131304 114131340

RMD:	113558345 113560783

BTAB:

DCI:

CLEC:

HHAL:

NLU:

PHU:	Phum_PHUM020230 Phum_PHUM022260

FOC:

113216433

TPAL:

117650592

ZNE:	110834052 110836715

CSEC:

111866672 111867140

FCD:	110845863 110848322

DPX:	DAPPUDRAFT_318471 DAPPUDRAFT_325909

DMK:

116919111

PVM:	113801041 113818841

PJA:	122256105 122262326

PCHN:

HAME:

PCLA:

PTRU:

HAZT:

108672861 108676913 108679476

LSM:

121120111

ISC:	IscW_ISCW009142 IscW_ISCW011416 IscW_ISCW022710

DSV:	119444686 119449573 119449574 119449654 119454033 119454036 119454045

RSAN:

119381061 119382543 119382544 119390585 119399038 119399203

RMP:	119163214 119163224 119177892 119179211 119179294 119186039

VDE:	111248006 111251269 111254734

VJA:	111261696 111262221 111263420

TUT:	107361061 107361357 107369198

DPTE:

113797543 113797547 113797548 113799106

DFR:	124490899 124490900 124496189

CSCU:

111620835 111621971 111630665 111636296 111636297

PTEP:

107444857 107447088 107447132

SDM:	118185055 118185056 118186253 118205799

HRO:	HELRODRAFT_116430 HELRODRAFT_119637 HELRODRAFT_186140 HELRODRAFT_96716

LGI:	LOTGIDRAFT_114212 LOTGIDRAFT_114214 LOTGIDRAFT_212916 LOTGIDRAFT_219759

BGT:

106077161

GAE:	121378475 121378607 121386093

HRF:	124111659 124111680 124122602 124132481 124132550 124132741 124142695 124146098 124146099

HRJ:	124263883 124264441 124264445 124268588 124279226 124279590 124290212

CRG:

MYI:

PMAX:

MMER:

OBI:

OSN:	115220794 115223218

LAK:	106161988 106164793 106171772 106174580

SMM:	Smp_020340 Smp_133210

SHX:	MS3_0013730

OVI:	T265_03531

EGL:

NVE:

EPA:

ATEN:

ADF:

AMIL:

PDAM:

SPIS:

DGT:

XEN:	124436700 124436776 124440224 124455773

HMG:	100200677 100211827

TAD:	TRIADDRAFT_10696 TRIADDRAFT_56702

AQU:

100634817

CVR:	CHLNCDRAFT_141480

SRE:	PTSG_12888

DDI:	DDB_G0269790(gaa)

DPP:	DICPUDRAFT_157944

DFA:	DFA_08363 DFA_12194(gaa)

ACAN:

ACA1_382370

PTM:	GSPATT00020305001 GSPATT00021096001 GSPATT00023547001

PIF:	PITG_10846

PSOJ:

PHYSODRAFT_472609

SPAR:

SPRG_04000 SPRG_09171

GTT:	GUITHDRAFT_114873

NGR:	NAEGRDRAFT_39431 NAEGRDRAFT_70309

» show all

Reference

PMID:23188827

Authors

Maga JA, Zhou J, Kambampati R, Peng S, Wang X, Bohnsack RN, Thomm A, Golata S, Tom P, Dahms NM, Byrne BJ, LeBowitz JH

Title

Glycosylation-independent lysosomal targeting of acid alpha-glucosidase enhances muscle glycogen clearance in pompe mice.

Journal

J Biol Chem 288:1428-38 (2013)
DOI:10.1074/jbc.M112.438663

Reference

PMID:24399866

Authors

Esmer C, Becerra-Becerra R, Pena-Zepeda C, Bravo-Oro A

Title

A novel homozygous mutation at the GAA gene in Mexicans with early-onset Pompe disease.

Journal

Acta Myol 32:95-9 (2013)

Sequence

[hsa:2548]

LinkDB

DBGET integrated database retrieval system