5NN3

Crystal structure of human lysosomal acid-alpha-glucosidase, GAA

Entity

Entity ID	Chain ID	Description	Type	Chain length	Formula weight	Number of molecules	DB Name (Accession)	Biological source	Descriptive keywords
1	A (A)	Lysosomal alpha-glucosidase	polymer	872	96978.6	1	UniProt (P10253) Pfam (PF00088) Pfam (PF13802) Pfam (PF01055) Pfam (PF21365)	Homo sapiens (Human)	Acid maltase,Aglucosidase alfa
2	B, F (B, F)	2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-[alpha-L-fucopyranose-(1-6)]2-acetamido-2-deoxy-beta-D-glucopyranose	branched		570.5	2	In PDB GlyTouCan (G21290RB)
3	C (C)	beta-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose	branched		586.5	1	In PDB GlyTouCan (G15407YE)
4	D (D)	2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose	branched		424.4	1	In PDB GlyTouCan (G42666HT)
5	E (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-glucopyranose	branched		748.7	1	In PDB GlyTouCan (G81315DD)
6	G, H (A)	SULFATE ION	non-polymer		96.1	2	Chemie (SO4)
7	I, J, K, L, M... (A)	CHLORIDE ION	non-polymer		35.5	8	Chemie (CL)
8	Q (A)	TRIETHYLENE GLYCOL	non-polymer		150.2	1	Chemie (PGE)
9	R (A)	DI(HYDROXYETHYL)ETHER	non-polymer		106.1	1	Chemie (PEG)
10	AA, BA, CA, S, T... (A)	1,2-ETHANEDIOL	non-polymer		62.1	11	Chemie (EDO)
11	DA (A)	water	water		18.0	668	Chemie (HOH)

Sequence modifications

A: 81 - 952 (UniProt: P10253)

PDB	External Database	Details
Arg 199	His 199	variant
His 223	Arg 223	variant
Ile 780	Val 780	variant

Contents of the asymmetric unit

Polymers	Number of chains	1
	Total formula weight	96978.6
Branched	Number of molecules	5
	Total formula weight	2900.7
Non-Polymers*	Number of molecules	23
	Total formula weight	1414.8
All*	Total formula weight	101294.1

*Water molecules are not included.