4DO4

Pharmacological chaperones for human alpha-N-acetylgalactosaminidase

Entity

Entity ID	Chain ID	Description	Type	Chain length	Formula weight	Number of molecules	DB Name (Accession)	Biological source	Descriptive keywords
1	A, B (A, B)	Alpha-N-acetylgalactosaminidase	polymer	400	45582.9	2	UniProt (P17050) Pfam (PF16499) Pfam (PF17450)	Homo sapiens (human)	Alpha-galactosidase B
2	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)
3	D, F (D, F)	alpha-D-mannopyranose-(1-3)-[alpha-D-mannopyranose-(1-6)]beta-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose	branched		910.8	2	In PDB GlyTouCan (G22768VO)
4	E (E)	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)
5	G, Q (A, B)	2-acetamido-2-deoxy-beta-D-glucopyranose	non-polymer		221.2	2	Chemie (NAG)
6	H, R (A, B)	N-[(3S,4R,5S,6R)-4,5-dihydroxy-6-(hydroxymethyl)piperidin-3-yl]acetamide	non-polymer		204.2	2	Chemie (DJN)
7	I, S (A, B)	CITRIC ACID	non-polymer		192.1	2	Chemie (CIT)
8	J, K, L, N, O... (A, B)	GLYCEROL	non-polymer		92.1	13	Chemie (GOL)
9	M (A)	ACETIC ACID	non-polymer		60.1	1	Chemie (ACY)
10	AA, BA (A, B)	water	water		18.0	991	Chemie (HOH)

Sequence modifications

A, B: 18 - 411 (UniProt: P17050)

PDB	External Database	Details
Gln 201	Asn 201	engineered mutation
His 412	-	expression tag
His 413	-	expression tag
His 414	-	expression tag
His 415	-	expression tag
His 416	-	expression tag
His 417	-	expression tag

Contents of the asymmetric unit

Polymers	Number of chains	2
	Total formula weight	91165.7
Branched	Number of molecules	4
	Total formula weight	2978.7
Non-Polymers*	Number of molecules	20
	Total formula weight	2492.4
All*	Total formula weight	96636.8

*Water molecules are not included.