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5NN5

Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with 1-deoxynojirimycin

Functional Information from GO Data
ChainGOidnamespacecontents
A0000023biological_processmaltose metabolic process
A0002026biological_processregulation of the force of heart contraction
A0002086biological_processdiaphragm contraction
A0003007biological_processheart morphogenesis
A0003824molecular_functioncatalytic activity
A0004553molecular_functionhydrolase activity, hydrolyzing O-glycosyl compounds
A0004558molecular_functionalpha-1,4-glucosidase activity
A0005764cellular_componentlysosome
A0005765cellular_componentlysosomal membrane
A0005886cellular_componentplasma membrane
A0005975biological_processcarbohydrate metabolic process
A0005977biological_processglycogen metabolic process
A0005980biological_processglycogen catabolic process
A0005985biological_processsucrose metabolic process
A0006006biological_processglucose metabolic process
A0006941biological_processstriated muscle contraction
A0007040biological_processlysosome organization
A0007626biological_processlocomotory behavior
A0009888biological_processtissue development
A0016020cellular_componentmembrane
A0016798molecular_functionhydrolase activity, acting on glycosyl bonds
A0030246molecular_functioncarbohydrate binding
A0035577cellular_componentazurophil granule membrane
A0035904biological_processaorta development
A0043181biological_processvacuolar sequestering
A0043202cellular_componentlysosomal lumen
A0043231cellular_componentintracellular membrane-bounded organelle
A0046716biological_processmuscle cell cellular homeostasis
A0050884biological_processneuromuscular process controlling posture
A0050885biological_processneuromuscular process controlling balance
A0060048biological_processcardiac muscle contraction
A0061723biological_processglycophagy
A0070062cellular_componentextracellular exosome
A0070821cellular_componenttertiary granule membrane
A0090599molecular_functionalpha-glucosidase activity
A0101003cellular_componentficolin-1-rich granule membrane
A0120282cellular_componentautolysosome lumen
Functional Information from PROSITE/UniProt
site_idPS00025
Number of Residues22
DetailsP_TREFOIL_1 P-type 'Trefoil' domain signature. RfdCaPdkaiTqeqCeargCCY
ChainResidueDetails
AARG89-TYR110

site_idPS00129
Number of Residues8
DetailsGLYCOSYL_HYDROL_F31_1 Glycosyl hydrolases family 31 active site. GMWiDMNE
ChainResidueDetails
AGLY514-GLU521

site_idPS00707
Number of Residues31
DetailsGLYCOSYL_HYDROL_F31_2 Glycosyl hydrolases family 31 signature 2. GADVCGFlgnTseeLCvRWtqLGAFyPFmRN
ChainResidueDetails
AGLY643-ASN673

Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues1
DetailsACT_SITE: Nucleophile => ECO:0000255|PROSITE-ProRule:PRU10066, ECO:0000269|PubMed:1856189, ECO:0000305|PubMed:29061980
ChainResidueDetails
AASP518

site_idSWS_FT_FI2
Number of Residues1
DetailsACT_SITE: ACT_SITE => ECO:0000250
ChainResidueDetails
AGLU521

site_idSWS_FT_FI3
Number of Residues4
DetailsBINDING: BINDING => ECO:0000305|PubMed:29061980
ChainResidueDetails
AASP404
AARG600
AASP616
AHIS674

site_idSWS_FT_FI4
Number of Residues2
DetailsCARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:29061980, ECO:0000269|PubMed:8435067, ECO:0000269|Ref.19, ECO:0007744|PDB:5KZW, ECO:0007744|PDB:5NN3
ChainResidueDetails
AASN140
AASN882

site_idSWS_FT_FI5
Number of Residues2
DetailsCARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:29061980, ECO:0000269|PubMed:8435067, ECO:0000269|Ref.19, ECO:0007744|PDB:5KZW, ECO:0007744|PDB:5NN3
ChainResidueDetails
AASN233
AASN652

site_idSWS_FT_FI6
Number of Residues1
DetailsCARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:16335952, ECO:0000269|PubMed:29061980, ECO:0000269|PubMed:8435067, ECO:0000269|Ref.19, ECO:0007744|PDB:5KZW, ECO:0007744|PDB:5NN3
ChainResidueDetails
AASN390

site_idSWS_FT_FI7
Number of Residues1
DetailsCARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:12754519, ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:29061980, ECO:0000269|PubMed:8435067, ECO:0000269|Ref.19, ECO:0007744|PDB:5KZW, ECO:0007744|PDB:5NN3
ChainResidueDetails
AASN470

site_idSWS_FT_FI8
Number of Residues1
DetailsCARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:8435067
ChainResidueDetails
AASN925

227561

PDB entries from 2024-11-20

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