Structure of PDB 5kzw Chain A

Receptor sequence
>5kzwA (length=850) Species: 9606 (Homo sapiens) [Search protein sequence]
PTQCDVPPNSRFDCAPDKAITQEQCEARGCCYIPAKQMGQPWCFFPPSYP
SYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIK
DPANRRYEVPLETPSPLYSVEFSEEPFGVIVHRQLDGRVLLNTTVAPLFF
ADQFLQLSTSLPSQYITGLAEHLSPLMLSTSWTRITLWNRDLAPTPGANL
YGSHPFYLALEDGGSAHGVFLLNSNAMDVVLQPSPALSWRSTGGILDVYI
FLGPEPKSVVQQYLDVVGYPFMPPYWGLGFHLCRWGYSSTAITRQVVENM
TRAHFPLDVQWNDLDYMDSRRDFTFNKDGFRDFPAMVQELHQGGRRYMMI
VDPAISSSGPAGSYRPYDEGLRRGVFITNETGQPLIGKVWPGSTAFPDFT
NPTALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIRGSEDGCPNNELENP
PYVPGVVGGTLQAATICASSHQFLSTHYNLHNLYGLTEAIASHRALVKAR
GTRPFVISRSTFAGHGRYAGHWTGDVWSSWEQLASSVPEILQFNLLGVPL
VGADVCGFLGNTSEELCVRWTQLGAFYPFMRNHNSLLSLPQEPYSFSEPA
QQAMRKALTLRYALLPHLYTLFHQAHVAGETVARPLFLEFPKDSSTWTVD
HQLLWGEALLITPVLQAGKAEVTGYFPLGTWYDLQTVPIEPREPAIHSEG
QWVTLPAPLDTINVHLRAGYIIPLQGPGLTTTESRQQPMALAVALTKGGE
ARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQ
KVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC
3D structure
PDB5kzw The structure of human GAA: structural basis of Pompe disease
ChainA
Resolution2.0 Å
3D
structure
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Enzymatic activity
Enzyme Commision number 3.2.1.20: alpha-glucosidase.
Interaction with ligand
Site
#		 Ligand Ligand
chain		 Binding residues on receptor
(original residue number in PDB)		 Binding residues on receptor
(residue number reindexed from 1)		 Binding affinity
BS01 GLC A W376 D404 L405 D518 R600 D616 F649 H674 W285 D313 L314 D427 R509 D525 F558 H583
BS02 BGC A W376 D404 L405 D518 R600 D616 F649 H674 W285 D313 L314 D427 R509 D525 F558 H583
BS03 GLC A D91 G123 Q124 P125 W126 C127 D13 G39 Q40 P41 W42 C43
BS04 BGC A A97 I98 W126 A19 I20 W42
Gene Ontology
Molecular Function
GO:0003824 catalytic activity
GO:0004553 hydrolase activity, hydrolyzing O-glycosyl compounds
GO:0004558 alpha-1,4-glucosidase activity
GO:0016798 hydrolase activity, acting on glycosyl bonds
GO:0030246 carbohydrate binding
GO:0090599 alpha-glucosidase activity
Biological Process
GO:0000023 maltose metabolic process
GO:0002026 regulation of the force of heart contraction
GO:0002086 diaphragm contraction
GO:0003007 heart morphogenesis
GO:0005975 carbohydrate metabolic process
GO:0005977 glycogen metabolic process
GO:0005980 glycogen catabolic process
GO:0005985 sucrose metabolic process
GO:0006006 glucose metabolic process
GO:0006941 striated muscle contraction
GO:0007040 lysosome organization
GO:0007626 locomotory behavior
GO:0009888 tissue development
GO:0035904 aorta development
GO:0043181 vacuolar sequestering
GO:0046716 muscle cell cellular homeostasis
GO:0050884 neuromuscular process controlling posture
GO:0050885 neuromuscular process controlling balance
GO:0060048 cardiac muscle contraction
GO:0061723 glycophagy
Cellular Component
GO:0005764 lysosome
GO:0005765 lysosomal membrane
GO:0005886 plasma membrane
GO:0016020 membrane
GO:0035577 azurophil granule membrane
GO:0043202 lysosomal lumen
GO:0043231 intracellular membrane-bounded organelle
GO:0070062 extracellular exosome
GO:0070821 tertiary granule membrane
GO:0101003 ficolin-1-rich granule membrane
GO:0120282 autolysosome lumen

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Molecular Function
View graph for
Biological Process
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Cellular Component
External links
PDB RCSB:5kzw, PDBe:5kzw, PDBj:5kzw
PDBsum5kzw
PubMed
UniProtP10253|LYAG_HUMAN Lysosomal alpha-glucosidase (Gene Name=GAA)

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