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9GSV

Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with iminosugar compound 4c

This is a non-PDB format compatible entry.
Entity
Entity IDChain IDDescriptionTypeChain lengthFormula weightNumber of moleculesDB Name (Accession)Biological sourceDescriptive keywords
1A
(A)
Lysosomal alpha-glucosidasepolymer952105448.51UniProt (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-L-fucopyranose-(1-6)]2-acetamido-2-deoxy-beta-D-glucopyranosebranched570.51In PDB
GlyTouCan (G21290RB)
3C, E
(C, E)
beta-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranosebranched586.52In 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)
5F
(F)
alpha-L-fucopyranose-(1-6)-2-acetamido-2-deoxy-beta-D-glucopyranosebranched367.31In PDB
GlyTouCan (G86851RC)
6G
(A)
(2~{R},3~{R},4~{R},5~{S})-2-(hydroxymethyl)-2-(2-phenylethyl)piperidine-3,4,5-triolnon-polymer267.31Chemie (A1IO6)
7H, I, J
(A)
SULFATE IONnon-polymer96.13Chemie (SO4)
8K, L, M, N, O
(A)
CHLORIDE IONnon-polymer35.55Chemie (CL)
9P, Q
(A)
TRIETHYLENE GLYCOLnon-polymer150.22Chemie (PGE)
10R, S
(A)
TETRAETHYLENE GLYCOLnon-polymer194.22Chemie (PG4)
11AA, BA, CA, T, U...
(A)
1,2-ETHANEDIOLnon-polymer62.110Chemie (EDO)
12DA
(A)
waterwater18.0475Chemie (HOH)
Sequence modifications
A: 1 - 952 (UniProt: P10253)
PDBExternal DatabaseDetails
Arg 199His 199variant
His 223Arg 223variant
Ile 780Val 780variant
Sequence viewer
Contents of the asymmetric unit
PolymersNumber of chains1
Total formula weight105448.5
BranchedNumber of molecules5
Total formula weight2535.4
Non-Polymers*Number of molecules23
Total formula weight2042.3
All*Total formula weight110026.1
*Water molecules are not included.

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PDB entries from 2026-03-11

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