5NN6

Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-hydroxyethyl-1-deoxynojirimycin

Entity

Entity ID	Chain ID	Description	Type	Chain length	Formula weight	Number of molecules	DB Name (Accession)	Biological source	Descriptive keywords
1	A	Lysosomal alpha-glucosidase	polymer	872	96978.6	1	UniProt (P10253) Pfam (PF00088) Pfam (PF13802) Pfam (PF01055) Pfam (PF21365) In PDB	Homo sapiens (Human)	Acid maltase,Aglucosidase alfa
2	B	2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-[alpha-L-fucopyranose-(1-6)]2-acetamido-2-deoxy-beta-D-glucopyranose	branched		570.5	1	In PDB GlyTouCan (G21290RB)
3	C, D	2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose	branched		424.4	2	In PDB GlyTouCan (G42666HT)
4	E	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)
5	F	alpha-L-fucopyranose-(1-6)-2-acetamido-2-deoxy-beta-D-glucopyranose	branched		367.3	1	In PDB GlyTouCan (G86851RC)
6	A	(2R,3R,4R,5S)-1-(2-hydroxyethyl)-2-(hydroxymethyl)piperidine-3,4,5-triol	non-polymer		207.2	1	Chemie (MIG)
7	A	SULFATE ION	non-polymer		96.1	5	Chemie (SO4)
8	A	CHLORIDE ION	non-polymer		35.5	3	Chemie (CL)
9	A	1,2-ETHANEDIOL	non-polymer		62.1	13	Chemie (EDO)
10	A	TRIETHYLENE GLYCOL	non-polymer		150.2	4	Chemie (PGE)
11	A	GLYCEROL	non-polymer		92.1	3	Chemie (GOL)
12		water	water		18.0	633	Chemie (HOH)

Sequence modifications

A: 81 - 952 (UniProt: P10253)

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

Contents of the asymmetric unit

Polymers	Number of chains	1
	Total formula weight	96978.6
Branched	Number of molecules	5
	Total formula weight	2373.2
Non-Polymers*	Number of molecules	29
	Total formula weight	2477.8
All*	Total formula weight	101829.6

*Water molecules are not included.