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5NN4

Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-acetyl-cysteine

Entity
Entity IDChain IDDescriptionTypeChain lengthFormula weightNumber of moleculesDB Name (Accession)Biological sourceDescriptive keywords
1A
(A)
Lysosomal alpha-glucosidasepolymer87296962.61UniProt (P10253)
Pfam (PF00088)
Pfam (PF13802)
Pfam (PF01055)
Pfam (PF21365)
Homo sapiens (Human)Acid maltase,Aglucosidase alfa
2B, F
(B, F)
2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-[alpha-L-fucopyranose-(1-6)]2-acetamido-2-deoxy-beta-D-glucopyranosebranched570.52In PDB
GlyTouCan (G21290RB)
3C
(C)
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)
4D
(D)
2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranosebranched424.41In PDB
GlyTouCan (G42666HT)
5E
(E)
alpha-D-mannopyranose-(1-3)-beta-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranosebranched748.71In PDB
GlyTouCan (G81315DD)
6G, H
(A)
N-ACETYL-L-CYSTEINEnon-polymer163.22Chemie (SC2)
7I, J, K, L
(A)
SULFATE IONnon-polymer96.14Chemie (SO4)
8M, N
(A)
CHLORIDE IONnon-polymer35.52Chemie (CL)
9O
(A)
TRIETHYLENE GLYCOLnon-polymer150.21Chemie (PGE)
10P, Q
(A)
GLYCEROLnon-polymer92.12Chemie (GOL)
11AA, BA, CA, DA, EA...
(A)
1,2-ETHANEDIOLnon-polymer62.118Chemie (EDO)
12JA
(A)
waterwater18.0776Chemie (HOH)
Sequence modifications
A: 81 - 952 (UniProt: P10253)
PDBExternal DatabaseDetails
Arg 199His 199conflict
His 223Arg 223conflict
Ile 780Val 780conflict
Sequence viewer
Contents of the asymmetric unit
PolymersNumber of chains1
Total formula weight96962.6
BranchedNumber of molecules5
Total formula weight2900.7
Non-Polymers*Number of molecules29
Total formula weight2233.1
All*Total formula weight102096.5
*Water molecules are not included.

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PDB entries from 2025-07-02

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