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7K66

Structure of Blood Coagulation Factor VIII in Complex with an Anti-C1 Domain Pathogenic Antibody Inhibitor

Entity
Entity IDChain IDDescriptionTypeChain lengthFormula weightNumber of moleculesDB Name (Accession)Biological sourceDescriptive keywords
1A
(A)
Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIIIpolymer1467168287.01UniProt (P12263)
UniProt (P00451)
Pfam (PF07732)
Pfam (PF00394)
Pfam (PF07731)
Pfam (PF00754)
Sus scrofa (Pig)Procoagulant component,Antihemophilic factor,AHF,Procoagulant component,Procoagulant component,Antihemophilic factor,AHF,Procoagulant component
2B
(B)
2A9 heavy chainpolymer22323979.81Mus musculus
3C
(C)
2A9 light chainpolymer21323297.71Mus musculus
4D
(H)
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-glucopyranosebranched910.81In PDB
GlyTouCan (G22768VO)
5E
(D)
2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranosebranched424.41In PDB
GlyTouCan (G42666HT)
6F
(A)
2-acetamido-2-deoxy-beta-D-glucopyranosenon-polymer221.21Chemie (NAG)
7G
(A)
COPPER (II) IONnon-polymer63.51Chemie (CU)
8H
(A)
CALCIUM IONnon-polymer40.11Chemie (CA)
9I
(A)
ZINC IONnon-polymer65.41Chemie (ZN)
10J
(B)
waterwater18.01Chemie (HOH)
Sequence modifications
A: -18 - 386 (UniProt: P12263)
A: 387 - 1626 (UniProt: P00451)
A: 1637 - 2019 (UniProt: P12263)
A: 2020 - 2332 (UniProt: P00451)
PDBExternal DatabaseDetails
Ala 1627-linker
Gln 1628-linker
Asn 1629-linker
Ser 1630-linker
Arg 1631-linker
Pro 1632-linker
Pro 1633-linker
Ser 1634-linker
Ala 1635-linker
Ser 1636-linker
Sequence viewer
Contents of the asymmetric unit
PolymersNumber of chains3
Total formula weight215564.5
BranchedNumber of molecules2
Total formula weight1335.2
Non-Polymers*Number of molecules4
Total formula weight390.2
All*Total formula weight217290.0
*Water molecules are not included.

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PDB entries from 2025-07-30

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