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5KZX

Crystal structure of human GAA

Entity
Entity IDChain IDDescriptionTypeChain lengthFormula weightNumber of moleculesDB Name (Accession)Biological sourceDescriptive keywords
1A
(A)
Lysosomal alpha-glucosidasepolymer87497141.81UniProt (P10253)
Pfam (PF00088)
Pfam (PF13802)
Pfam (PF01055)
Pfam (PF21365)
Homo sapiens (Human)Acid maltase,Aglucosidase alfa
2B
(B)
2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-[alpha-D-fucopyranose-(1-6)]2-acetamido-2-deoxy-beta-D-glucopyranosebranched570.51In PDB
GlyTouCan (G38216KS)
3C, D
(C, D)
2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranosebranched424.42In PDB
GlyTouCan (G42666HT)
4E
(E)
beta-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranosebranched586.51In PDB
GlyTouCan (G15407YE)
5F
(F)
alpha-D-fucopyranose-(1-6)-2-acetamido-2-deoxy-beta-D-glucopyranosebranched367.31In PDB
GlyTouCan (G18989MS)
6G
(A)
2-acetamido-2-deoxy-beta-D-glucopyranosenon-polymer221.21Chemie (NAG)
7H, J
(A)
alpha-D-glucopyranosenon-polymer180.22Chemie (GLC)
8I, K
(A)
beta-D-glucopyranosenon-polymer180.22Chemie (BGC)
9L, M, T
(A)
1,2-ETHANEDIOLnon-polymer62.13Chemie (EDO)
10N
(A)
TRIETHYLENE GLYCOLnon-polymer150.21Chemie (PGE)
11O, R
(A)
DI(HYDROXYETHYL)ETHERnon-polymer106.12Chemie (PEG)
12P, Q, S
(A)
SULFATE IONnon-polymer96.13Chemie (SO4)
13U
(A)
waterwater18.0792Chemie (HOH)
Sequence modifications
A: 79 - 952 (UniProt: P10253)
PDBExternal DatabaseDetails
His 223Arg 223conflict
Ile 780Val 780conflict
Sequence viewer
Contents of the asymmetric unit
PolymersNumber of chains1
Total formula weight97141.8
BranchedNumber of molecules5
Total formula weight2373.2
Non-Polymers*Number of molecules14
Total formula weight1778.6
All*Total formula weight101293.7
*Water molecules are not included.

246704

PDB entries from 2025-12-24

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