KEGG ORTHOLOGY: K12316

ORTHOLOGY: K12316

Entry

K12316 KO

Name

GAA

Definition

lysosomal alpha-glucosidase [EC:3.2.1.20]

Pathway

ko00052

Galactose metabolism

ko00500

Starch and sucrose metabolism

ko01100

Metabolic pathways

ko01110

Biosynthesis of secondary metabolites

ko04142

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)

RRO:	104675242(GAA)

RBB:	108534154(GAA)

TFN:	117066796(GAA)

CJC:	100411176(GAA)

SBQ:	101042074(GAA)

MMUR:

105866728(GAA)

MMU:	14387(Gaa)

MCAL:

110305854(Gaa)

MPAH:

110331633(Gaa)

RNO:	367562(Gaa)

MCOC:

116077227(Gaa)

MUN:	110550113(Gaa)

CGE:	100755105(Gaa)

PLEU:

114687821(Gaa)

NGI:	103730596(Gaa)

HGL:	101718861(Gaa)

CCAN:

109684706(Gaa)

OCU:	103347590(GAA)

TUP:	102474055(GAA)

CFA:	483352(GAA)

VVP:	112920872(GAA)

AML:	100477070(GAA)

UMR:	103665978(GAA)

UAH:	113244540(GAA)

ORO:	101386503(GAA)

ELK:

111160434

MPUF:

101689681(GAA)

EJU:	114216122(GAA)

FCA:	101086359(GAA)

PTG:	102956310(GAA)

PPAD:

109276817(GAA)

AJU:	106981879(GAA)

BTA:	280798(GAA)

BOM:	102273281(GAA)

BIU:	109574062(GAA)

BBUB:

102407679(GAA)

CHX:	102175798(GAA)

OAS:	101121692(GAA)

SSC:	100526132(GAA)

CFR:	102510030(GAA)

CDK:	105106261(GAA)

BACU:

103019416(GAA)

LVE:	103090683(GAA)

OOR:	101288921(GAA)

DLE:	111182733(GAA)

PCAD:

102978552(GAA)

ECB:	100056675(GAA)

EPZ:	103547327(GAA)

EAI:	106845535(GAA)

MYB:	102264084(GAA)

MYD:	102751832(GAA)

MMYO:

118672068(GAA)

MNA:	107532060(GAA)

HAI:	109373269 109383724(GAA)

DRO:	112298701(GAA)

SHON:

118999415(GAA)

AJM:	119058623(GAA)

MMF:	118634780(GAA)

PALE:

102885829(GAA)

RAY:	107510082(GAA)

MJV:	108387035(GAA)

LAV:	100659459(GAA)

TMU:

101344373

MDO:	100028995 100030818(GAA)

SHR:	100914209(GAA) 100933026

PCW:	110200106 110218205(GAA)

OAA:	100079324(GAA) 100083052

GGA:	416462(GAAGSD) 422082(GAA)

MGP:	100547897 100548976(GAA)

CJO:	107320667 107321973(GAA)

NMEL:

110406141 110407405(GAA)

APLA:

113845291

ACYG:

106033539 106046740(GAA)

TGU:	100226089 100227842(GAA)

LSR:	110472472(GAA) 110475464

SCAN:

103817641 103819935(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)

FPG:	101918651(GAA) 101924021

FCH:	102046260 102051071(GAA)

CLV:	102090362(GAA) 102090839

EGZ:	104128958 104130629(GAA)

NNI:	104014328(GAA)

ACUN:

113485633

PADL:

103913521

AAM:	106491310 106500263

ASN:	102379461 102381953(GAA)

AMJ:	102572123 102577078(GAA)

PSS:	102459990(GAA)

CMY:	102934350 102939927(GAA)

CPIC:

101932947 101945048(GAA)

ACS:	100553663 100561813(gaa)

PVT:	110084704 110090137(GAA)

PBI:	103061161 103065099(GAA)

PMUR:

107288787(GAA) 107290641

TSR:	106544831 106553676(GAA)

PMUA:

114583983 114589000(GAA)

GJA:	107118778 107119854(GAA)

XLA:

108703274

XTR:	100495955(gaa)

NPR:	108792966 108792971

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

IPU:	108258846 108260916(gaa)

PHYP:

113528512(gaa) 113535694

AMEX:

103033787 103044115(gaa)

EEE:	113573276(gaa) 113574921

TRU:	101074630 101076752 105419329

TNG:	GSTEN00015697G001 GSTEN00033404G001

LCO:	104918857(gaa) 104926848

NCC:	104949822 104964793(gaa)

MZE:	101467306(gaa) 101478649

ONL:	100691577(gaa) 100695517

OLA:	101161640(gaa) 101169174

XMA:	102223585 102231710(gaa)

XCO:	114144344 114160012(gaa)

PRET:

103469228(gaa) 103481270

CVG:	107092809(gaa) 107097057

NFU:	107376530(gaa) 107389057

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

ALIM:

106518748(gaa) 106522707

AOCE:

111577359(gaa) 111588140

CSEM:

103383270 103393066(gaa)

POV:	109638725(gaa) 109642103

LCF:	108879632 108887206 108894833

SDU:	111216602 111227137(gaa)

SLAL:

111655551 111667512(gaa)

HCQ:	109511032 109522721(gaa)

BPEC:

110157747 110166448(gaa)

MALB:

109952354 109969497(gaa)

SASA:

106564132 106588841 106595381 106601771

OTW:	112259065(gaa)

SALP:

112073863

ELS:	105013030(gaa) 105029840

SFM:	108926133 108936742(gaa)

PKI:	111843280(gaa) 111845470

LCM:	102355272(GAA) 102366792

CMK:	103175511(gaa) 103186597

RTP:	109926703 109931212 109931213

BFO:	118427978 118427981 118429186

CIN:

100187282

SPU:	582502 764550

APLC:

110986134 110986153 110986154

SKO:	100366661 100371743 100372586 102804585

AGA:	AgaP_AGAP001534

AAG:

AALB:

AME:

BIM:

BTER:

CCAL:

OBB:

SOC:

MPHA:

AEC:

ACEP:

PBAR:

VEM:

HST:

DQU:

CFO:

LHU:

PGC:

OBO:

PCF:

NVI:	100121715 100124137

CSOL:

105366539

MDL:

103577431

TCA:	657390 658192

DPA:	109537673 109539233

ATD:

109598697

NVL:	108561339 108567530

BMOR:

101736905 101744138

BMAN:

114243285 114248263

DPL:	KGM_204829 KGM_212394

PMAC:

106718904

PRAP:

110995043 111003643

HAW:	110373713 110384254

TNL:	113497839 113503307

PXY:

105385777

API:	100158725 100160797

DNX:	107169248 107169249

AGS:	114131304 114131340

RMD:	113558345 113560783

BTAB:

109033364 109040182

CLEC:

106663068

PHU:	Phum_PHUM020230 Phum_PHUM022260

ZNE:	110834052 110836715

FCD:	110845863 110848322

DPX:	DAPPUDRAFT_318471 DAPPUDRAFT_325909

PVM:	113801041 113818841

ISC:	IscW_ISCW009142 IscW_ISCW011416 IscW_ISCW022710

TUT:	107361061 107361357 107369198

DPTE:

113797543 113797547 113797548 113799106

CSCU:

111620835 111621971 111630665 111636296 111636297

PTEP:

107444857 107447088 107447132

HRO:	HELRODRAFT_116430 HELRODRAFT_119637 HELRODRAFT_186140 HELRODRAFT_96716

LGI:	LOTGIDRAFT_114212 LOTGIDRAFT_114214 LOTGIDRAFT_212916 LOTGIDRAFT_219759

CRG:

105328336

MYI:

110466933

OBI:

106873500

LAK:	106161988 106164793 106171772 106174580

SMM:	Smp_020340 Smp_133210

OVI:	T265_03531

EGL:

NVE:

EPA:

ADF:

AMIL:

PDAM:

SPIS:

DGT:

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