ORTHOLOGY: K12316
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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
BRITE hierarchy
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:
120950793
AARA:
120896393
ASTE:
118505663
AAG:
23687646
AALB:
109418271
109428050
CPII:
120432378
AME:
409365
ACER:
108000607
ALAB:
122713254
BIM:
100745310
BBIF:
117212881
BVK:
117237021
BVAN:
117162501
BTER:
100650486
BPYO:
122571158
CCAL:
108623473
OBB:
114878375
MGEN:
117220176
NMEA:
116428120
CGIG:
122396934
SOC:
105196322
MPHA:
105831235
AEC:
105145111
ACEP:
105623984
PBAR:
105429028
VEM:
105569993
HST:
105181904
DQU:
106748183
CFO:
105248635
FEX:
115234617
LHU:
105674439
PGC:
109856932
OBO:
105286746
PCF:
106792256
PFUC:
122513907
VPS:
122633694
NVI:
100121715
100124137
CSOL:
105366539
TPRE:
106658190
MDL:
103577431
CGLO:
123264385
FAS:
105264204
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:
109033364
109040182
DCI:
103519355
CLEC:
106663068
HHAL:
106688304
NLU:
111055492
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:
125033514
125035872
HAME:
121869389
121872613
PCLA:
123747632
123759401
PTRU:
123506837
123519351
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:
105328336
MYI:
110466933
PMAX:
117325327
MMER:
123564304
123564789
OBI:
106873500
OSN:
115220794
115223218
LAK:
106161988
106164793
106171772
106174580
SMM:
Smp_020340
Smp_133210
SHX:
MS3_0013730
OVI:
T265_03531
EGL:
EGR_08499
NVE:
5513664
EPA:
110237636
ATEN:
116308764
ADF:
107356546
AMIL:
114958834
PDAM:
113670787
SPIS:
111336128
DGT:
114527280
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
Taxonomy
UniProt
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
]
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