7k66

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

Method: X-RAY DIFFRACTION D_max: 153.4 Å Quality: GOOD

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1. Protein Identity and Related Structures Protein Identity & Related Structures

Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII

Homo sapiens

State in the Current Structure

Assembly	Oligomeric State	Construct	Mutations and Modifications	Ligands, Ions and Associated Components	Method and Experimental Conditions	Structure Quality
1	Insufficient information Heteromer Protein × 3 其他Polymer 2 PDB declaration: trimeric（3） Consistent with protein copy count	Chain A； UniProt 406–761 Chain A； UniProt 2039–2351	Not recorded	2A9 heavy chain × 1 2A9 light chain × 1 ;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 ; × 1 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose × 1 NAG 2-acetamido-2-deoxy-beta-D-glucopyranose × 1 CU COPPER (II) ION × 1 CA CALCIUM ION × 1 ZN ZINC ION × 1	X-RAY DIFFRACTION X-ray crystallization conditions：VAPOR DIFFUSION, HANGING DROP；pH 7.7；293 K；17.5 %(w/v) PEG 1500, 50 mM HEPES (pH 7.7)	Resolution 3.92 Å R-free 0.322

Other States of the Same Protein in the Database

Each row is a biological assembly of the same UniProt protein in another PDB entry. The “Difference from current entry” column identifies evidence-level differences; no tag means the currently parsed fields agree.

Other PDB	Difference from Current Entry 7K66	Assembly / Oligomeric State	Construct	Mutations and Modifications	Ligands, Ions and Non-polymers	Method and Experimental Conditions	Structure Quality
12JP Structural determination of lipid-bound Factor VIII Deposited 2026-04-08	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain A 20–2351（2332 aa）	Not recorded	No recorded non-water small molecule	ELECTRON MICROSCOPY mmCIF provides none of the parsed conditions	Resolution 3.46 Å
12JV Structural determination of lipid-bound Factor VIII Deposited 2026-04-08	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain A 20–2351（2332 aa）	Not recorded	No recorded non-water small molecule	ELECTRON MICROSCOPY mmCIF provides none of the parsed conditions	Resolution 3.56 Å
1CFG MEMBRANE-BINDING PEPTIDE FROM THE C2 DOMAIN OF FACTOR VIII FORMS AN AMPHIPATHIC STRUCTURE AS DETERMINED BY NMR SPECTROSCOPY Deposited 1994-11-10	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain A 2322–2343（22 aa）	Not recorded	No recorded non-water small molecule	SOLUTION NMR mmCIF provides none of the parsed conditions	Resolution not provided
1D7P Crystal structure of the c2 domain of human factor viii at 1.5 a resolution at 1.5 A Deposited 1999-10-19	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain M 2190–2348（159 aa） Fragment：C2-DOMAIN	Mutation：S2296C	SO4 SULFATE ION × 2 CYS CYSTEINE × 1 GOL GLYCEROL × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, SITTING DROP；pH 6.5；298 K；AMMONIUM SULFATE, pH 6.5, VAPOR DIFFUSION, SITTING DROP, temperature 298.0K	Resolution 1.50 Å R-free 0.215
1FAC COAGULATION FACTOR VIII, NMR, 1 STRUCTURE Deposited 1996-06-30	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain A 2322–2342（21 aa）	Not recorded	No recorded non-water small molecule	SOLUTION NMR NMR measurement conditions pH 4；308.3 K	Resolution not provided
1IQD Human Factor VIII C2 Domain complexed to human monoclonal BO2C11 Fab. Deposited 2001-07-21	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein heterocomplex Heteromer；Protein × 3 PDB declaration: trimeric	Chain C 2193–2348（156 aa） Fragment：C-TERMINAL DOMAIN	Mutation：S2296C	No recorded non-water small molecule	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 7；298 K；PEG8000, sodium chloride, HEPES, pH 7.0, VAPOR DIFFUSION, HANGING DROP, temperature 298K	Resolution 2.00 Å R-free 0.253
2R7E Crystal Structure Analysis of Coagulation Factor VIII Deposited 2007-09-07	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Other combination Homooligomer；Protein × 2 PDB declaration: dimeric	Chain A 19–760（742 aa） Fragment：residues 19-760 Chain B 1582–2351（770 aa） Fragment：residues 1582-2351	Mutation：F1838S	CA CALCIUM ION × 3 CU COPPER (II) ION × 2	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 7.5；277 K；8-10% PEG 8000, 0.1M Imidazole, 100-300 mM NaCl, pH 7.5, VAPOR DIFFUSION, HANGING DROP, temperature 277K	Resolution 3.70 Å R-free 0.347
3CDZ Crystal structure of human factor VIII Deposited 2008-02-27	Different construct Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Other combination Homooligomer；Protein × 2 PDB declaration: dimeric	Chain A 20–759（740 aa） Fragment：B-domain deleted (UNP residues 20-764) Chain B 1668–2351（684 aa） Fragment：B-domain deleted (UNP residues 1668-2351)	Not recorded	CU COPPER (II) ION × 2 CA CALCIUM ION × 1 NAG 2-acetamido-2-deoxy-beta-D-glucopyranose × 2	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 8.5；298 K；100 mM Tris-HCl, 10% EtOH, 7% PEG 3350, pH 8.5, VAPOR DIFFUSION, HANGING DROP, temperature 298K	Resolution 3.98 Å R-free 0.327
3HNB Factor VIII Trp2313-His2315 segment is involved in membrane binding as shown by crystal structure of complex between factor VIII C2 domain and an inhibitor Deposited 2009-05-31	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain M 2189–2347（159 aa） Fragment：C2 domain of Factor VIIIa light chain, UNP residues 2189-2347	Not recorded	768 (2R)-1-(2,4-dichlorophenoxy)-3-[(2E)-2-imino-3-(2-piperidin-1-ylethyl)-2,3-dihydro-1H-benzimidazol-1-yl]propan-2-ol × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 8；298 K；2.8M sodium chloride, 0.1M Tris-HCl, 3% glycol, pH 8.0, VAPOR DIFFUSION, HANGING DROP, temperature 298K	Resolution 1.15 Å R-free 0.220
3HNY Factor VIII Trp2313-His2315 segment is involved in membrane binding as shown by crystal structure of complex between factor VIII C2 domain and an inhibitor Deposited 2009-06-01	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain M 2189–2347（159 aa） Fragment：factor VIII c2 domain	Not recorded	No recorded non-water small molecule	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 8；298 K；2.8M sodium chloride, 0.1M Tris-HCl, 3% glycol, pH 8.0, VAPOR DIFFUSION, HANGING DROP, temperature 298K	Resolution 1.07 Å R-free 0.201
3HOB Factor VIII Trp2313-His2315 segment is involved in membrane binding as shown by crystal structure of complex between factor VIII C2 domain and an inhibitor Deposited 2009-06-01	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain M 2189–2347（159 aa） Fragment：factor VIII c2 domain	Not recorded	No recorded non-water small molecule	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, SITTING DROP；pH 6；298 K；30.0% PEG 2000, MME 0.15M KBr, pH 6.0, VAPOR DIFFUSION, SITTING DROP, temperature 298K	Resolution 2.07 Å R-free 0.263
3HOB Factor VIII Trp2313-His2315 segment is involved in membrane binding as shown by crystal structure of complex between factor VIII C2 domain and an inhibitor Deposited 2009-06-01	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 2 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain A 2189–2347（159 aa） Fragment：factor VIII c2 domain	Not recorded	No recorded non-water small molecule	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, SITTING DROP；pH 6；298 K；30.0% PEG 2000, MME 0.15M KBr, pH 6.0, VAPOR DIFFUSION, SITTING DROP, temperature 298K	Resolution 2.07 Å R-free 0.263
3J2Q Model of membrane-bound factor VIII organized in 2D crystals Deposited 2012-12-11	Different construct Different oligomeric state Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Other combination Homooligomer；Protein × 2 PDB declaration: dimeric	Chain A 20–764（745 aa） Fragment：UNP residues 20-764 Chain B 1668–2351（684 aa） Fragment：UNP residues 1668-2351	Not recorded	CU COPPER (II) ION × 2 CA CALCIUM ION × 1 NAG 2-acetamido-2-deoxy-beta-D-glucopyranose × 2	ELECTRON CRYSTALLOGRAPHY mmCIF provides none of the parsed conditions	Resolution 15.00 Å
3J2S Membrane-bound factor VIII light chain Deposited 2012-12-21	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain B 1710–2351（642 aa） Fragment：UNP residues 1710-2356	Not recorded	No recorded non-water small molecule	ELECTRON MICROSCOPY cryo-EM buffer 20 mM Tris-HCl, 150 mM NaCl, 5mM CaCl2；pH 7.4；20 mM Tris-HCl, 150 mM NaCl, 5mM CaCl2 cryo-EM vitrification conditions Blot for 3.5 seconds before plunging；95 K；Cryogen ETHANE；Blot for 3.5 seconds before plunging into liquid ethane (Vitrobot Mark III)	Resolution 15.00 Å
4BDV CRYSTAL STRUCTURE OF A TRUNCATED B-DOMAIN HUMAN FACTOR VIII Deposited 2012-10-08	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Other combination Homooligomer；Protein × 2 PDB declaration: dimeric	Chain A 20–769（750 aa） Fragment：COAGULATION FACTOR VIII, RESIDUES 20-769,1657-1666 Chain A 1657–1666（10 aa） Fragment：COAGULATION FACTOR VIII, RESIDUES 20-769,1657-1666 Chain B 1667–2351（685 aa） Fragment：RESIDUES 1667-2351	Not recorded	ZN ZINC ION × 1 CA CALCIUM ION × 1 EDO 1,2-ETHANEDIOL × 2 CU1 COPPER (I) ION × 1	X-RAY DIFFRACTION X-ray crystallization conditions pH 7.5；3% PEG550, 0.100 M NACL, 0.100 M TRIS-HCL PH 7.5, 10% ETHANOL	Resolution 3.98 Å R-free 0.246
4KI5 Cystal structure of human factor VIII C2 domain in a ternary complex with murine inhbitory antibodies 3E6 and G99 Deposited 2013-05-01	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein heterocomplex Heteromer；Protein × 5 PDB declaration: pentameric	Chain M 2190–2351（162 aa） Fragment：C2 domain	Not recorded	No recorded non-water small molecule	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 7.5；293 K；28% PEG-1000, 0.15 M sodium acetate, 0.025 M Tris-HCl, 0.05 M NaCl, 0.15 M ammonium nitrate, pH 7.5, VAPOR DIFFUSION, HANGING DROP, temperature 293K	Resolution 2.47 Å R-free 0.238
4PT6 The discobody: an engineered discoidin domain from factor VIII that binds v 3 integrin with antibody-like affinities Deposited 2014-03-10	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain A 2190–2351（162 aa） Fragment：unp residues 2190-2351	Not recorded	No recorded non-water small molecule	X-RAY DIFFRACTION mmCIF provides none of the parsed conditions	Resolution 2.10 Å R-free 0.252
4PT6 The discobody: an engineered discoidin domain from factor VIII that binds v 3 integrin with antibody-like affinities Deposited 2014-03-10	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 2 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain B 2190–2351（162 aa） Fragment：unp residues 2190-2351	Not recorded	No recorded non-water small molecule	X-RAY DIFFRACTION mmCIF provides none of the parsed conditions	Resolution 2.10 Å R-free 0.252
4XZU Crystal Structure of the Human Factor VIII C2 Domain in Complex with Murine 3E6 Inhibitory Antibody Deposited 2015-02-05	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein heterocomplex Heteromer；Protein × 3 PDB declaration: trimeric	Chain M 2193–2346（154 aa）	Not recorded	MG MAGNESIUM ION × 2	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 6；298 K；12-18% PEG 8000, MES, 0-200 mM magnesium chloride	Resolution 2.61 Å R-free 0.267
4XZU Crystal Structure of the Human Factor VIII C2 Domain in Complex with Murine 3E6 Inhibitory Antibody Deposited 2015-02-05	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 2 Protein heterocomplex Heteromer；Protein × 3 PDB declaration: trimeric	Chain G 2193–2346（154 aa）	Not recorded	MG MAGNESIUM ION × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 6；298 K；12-18% PEG 8000, MES, 0-200 mM magnesium chloride	Resolution 2.61 Å R-free 0.267
5K8D Crystal structure of rFVIIIFc Deposited 2016-05-29	Different construct Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Insufficient information Heteromer；Protein × 2 PDB declaration: dimeric	Chain A 20–759（740 aa） Chain B 1713–2350（638 aa）	Not recorded	CA CALCIUM ION × 3 CU COPPER (II) ION × 2	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION；pH 7.3；297 K；5% Ethanol, 100mM TRIS pH 7.3	Resolution 4.19 Å R-free 0.362
6MF0 Crystal Structure Determination of Human/Porcine Chimera Coagulation Factor VIII Deposited 2018-09-07	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Insufficient information Homooligomer；Protein × 2 PDB declaration: monomeric	Chain A 387–761（375 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain A 2039–2351（313 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain B 387–761（375 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain B 2039–2351（313 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human)	Not recorded	CA CALCIUM ION × 2 ZN ZINC ION × 2 CU1 COPPER (I) ION × 2	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；293 K；16% PEG3350, 0.025 M magnesium chloride, 0.025 M HEPES	Resolution 3.20 Å R-free 0.287
6MF0 Crystal Structure Determination of Human/Porcine Chimera Coagulation Factor VIII Deposited 2018-09-07	Different construct Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 2 Insufficient information Monomer；Protein × 1 PDB declaration: monomeric	Chain A 387–761（375 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain A 2039–2351（313 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human)	Not recorded	CA CALCIUM ION × 1 ZN ZINC ION × 1 CU1 COPPER (I) ION × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；293 K；16% PEG3350, 0.025 M magnesium chloride, 0.025 M HEPES	Resolution 3.20 Å R-free 0.287
6MF2 Improved Model of Human Coagulation Factor VIII Deposited 2018-09-08	Different construct Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Other combination Monomer；Protein × 1 PDB declaration: monomeric	Chain A 19–760（742 aa） Fragment：UNP residues 19-760,1582-2351 Chain A 1582–2351（770 aa） Fragment：UNP residues 19-760,1582-2351	Not recorded	CA CALCIUM ION × 1 ZN ZINC ION × 1 CU1 COPPER (I) ION × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 7.5；277 K；8-10% PEG8000, 0.1 M imidazole, 100-300 mM sodium chloride	Resolution 3.61 Å R-free 0.284
7KBT Factor VIII in complex with the anti-C2 domain antibody, G99 Deposited 2020-10-02	Different construct Different experimental conditions Different structure-quality metrics	Assembly 1 Insufficient information Heteromer；Protein × 3 PDB declaration: trimeric	Chain A 387–761（375 aa） Chain A 2039–2351（313 aa）	Not recorded	NAG 2-acetamido-2-deoxy-beta-D-glucopyranose × 1 CU COPPER (II) ION × 1 ZN ZINC ION × 1 CA CALCIUM ION × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；293 K；8 - 18% PEG 1500, PEG 6K, or PEG 10K and 50mM malic acid, pH 7.	Resolution 4.15 Å R-free 0.338
8G6I Coagulation factor VIII bound to a patient-derived anti-C1 domain antibody inhibitor Deposited 2023-02-15	Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Insufficient information Heteromer；Protein × 3 PDB declaration: trimeric	Chain A 406–761（356 aa） Chain A 2039–2351（313 aa）	Not recorded	No recorded non-water small molecule	ELECTRON MICROSCOPY cryo-EM buffer pH 7.4；150 mM NaCl, 20 mM HEPES pH 7.4 cryo-EM vitrification conditions Cryogen ETHANE	Resolution 4.23 Å
8TY1 Cryo-EM structure of coagulation factor VIII bound to NB2E9 Deposited 2023-08-24	Different construct Different oligomeric state Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Insufficient information Heteromer；Protein × 3 PDB declaration: trimeric	Chain A 392–771（380 aa） Chain A 2039–2351（313 aa）	Not recorded	No recorded non-water small molecule	ELECTRON MICROSCOPY cryo-EM buffer pH 7.4；20 mM HEPES (pH 7.4) and 150 mM NaCl cryo-EM vitrification conditions Cryogen ETHANE	Resolution 3.46 Å
9D5D Crystal Structure of Blood Coagulation Factor VIII C2 Domain Mutant L2251A/L2252A Deposited 2024-08-13	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain M 2191–2351（161 aa） Fragment：C2 domain (UNP residues 2191-2351)	Mutation：L2251A,L2252A	No recorded non-water small molecule	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；298 K；4 M potassium formate, 0.1 M Bis-Tris propane, pH 9, 2% w/v PEG2000 MME	Resolution 1.83 Å R-free 0.223
9JBV Structure of Engineered Coagulation Factor VIII with Enhanced Secretion and Coagulation Potential Deposited 2024-08-27	Different construct Different oligomeric state Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain A 1–764（764 aa） Chain A 1659–2351（693 aa）	Not recorded	No recorded non-water small molecule	ELECTRON MICROSCOPY cryo-EM buffer pH 7.3 cryo-EM vitrification conditions Cryogen ETHANE	Resolution 3.17 Å

25 other PDB entries and 29 assemblies. Open the comparison page and filter oligomeric states

View Construct and Data Evidence

UniProt name	FA8_HUMAN
Isoform	—
PDB entities	1
Chains and sequence ranges	Author chain A； PDBConstruct 406–761； UniProt 406–761 Author chain A； PDBConstruct 1155–1467； UniProt 2039–2351

Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII

Homo sapiens

UniProt P12263

State in the Current Structure

Assembly	Oligomeric State	Construct	Mutations and Modifications	Ligands, Ions and Associated Components	Method and Experimental Conditions	Structure Quality
1	Insufficient information Heteromer Protein × 3 其他Polymer 2 PDB declaration: trimeric（3） Consistent with protein copy count	Chain A； UniProt 1–405 Chain A； UniProt 1438–1820	Not recorded	2A9 heavy chain × 1 2A9 light chain × 1 ;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 ; × 1 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose × 1 NAG 2-acetamido-2-deoxy-beta-D-glucopyranose × 1 CU COPPER (II) ION × 1 CA CALCIUM ION × 1 ZN ZINC ION × 1	X-RAY DIFFRACTION X-ray crystallization conditions：VAPOR DIFFUSION, HANGING DROP；pH 7.7；293 K；17.5 %(w/v) PEG 1500, 50 mM HEPES (pH 7.7)	Resolution 3.92 Å R-free 0.322

Other States of the Same Protein in the Database

Other PDB	Difference from Current Entry 7K66	Assembly / Oligomeric State	Construct	Mutations and Modifications	Ligands, Ions and Non-polymers	Method and Experimental Conditions	Structure Quality
4MO3 Crystal Structure of Porcine C2 Domain of Blood Coagulation Factor VIII Deposited 2013-09-11	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain M 1971–2129（159 aa） Fragment：C2 domain (UNP residues 1971-2129)	Not recorded	GOL GLYCEROL × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；pH 10.4；277 K；2:1 crystallization buffer (0.1 M magnesium acetate, 10% ethanol) to protein, crystals formed overnight with 0.1 M CHES buffer and 220 uL Hampton Research Al's oil over 400 uL reservoir, pH 10.4, VAPOR DIFFUSION, HANGING DROP, temperature 277K	Resolution 1.70 Å R-free 0.209
6MF0 Crystal Structure Determination of Human/Porcine Chimera Coagulation Factor VIII Deposited 2018-09-07	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Insufficient information Homooligomer；Protein × 2 PDB declaration: monomeric	Chain A 1–386（386 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain A 762–771（10 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain A 1438–1820（383 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain B 1–386（386 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain B 762–771（10 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain B 1438–1820（383 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human)	Not recorded	CA CALCIUM ION × 2 ZN ZINC ION × 2 CU1 COPPER (I) ION × 2	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；293 K；16% PEG3350, 0.025 M magnesium chloride, 0.025 M HEPES	Resolution 3.20 Å R-free 0.287
6MF0 Crystal Structure Determination of Human/Porcine Chimera Coagulation Factor VIII Deposited 2018-09-07	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 2 Insufficient information Monomer；Protein × 1 PDB declaration: monomeric	Chain A 1–386（386 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain A 762–771（10 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human) Chain A 1438–1820（383 aa） Fragment：UNP residues 1-386 (pig), 387-761 (human), 762-771,1438-1820 (pig), 2039-2351 (human)	Not recorded	CA CALCIUM ION × 1 ZN ZINC ION × 1 CU1 COPPER (I) ION × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；293 K；16% PEG3350, 0.025 M magnesium chloride, 0.025 M HEPES	Resolution 3.20 Å R-free 0.287
7KBT Factor VIII in complex with the anti-C2 domain antibody, G99 Deposited 2020-10-02	Different construct Different mutation/modification Different experimental conditions Different structure-quality metrics	Assembly 1 Insufficient information Heteromer；Protein × 3 PDB declaration: trimeric	Chain A 1–386（386 aa） Chain A 762–771（10 aa） Chain A 1438–1820（383 aa）	Not recorded	NAG 2-acetamido-2-deoxy-beta-D-glucopyranose × 1 CU COPPER (II) ION × 1 ZN ZINC ION × 1 CA CALCIUM ION × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；293 K；8 - 18% PEG 1500, PEG 6K, or PEG 10K and 50mM malic acid, pH 7.	Resolution 4.15 Å R-free 0.338
7S0P Crystal structure of Porcine Factor VIII C2 Domain Bound to Phosphatidylserine Deposited 2021-08-30	Different construct Different mutation/modification Different oligomeric state Different ligand/ion Different experimental conditions Different structure-quality metrics	Assembly 1 Protein monomer Monomer；Protein × 1 PDB declaration: monomeric	Chain M 1974–2129（156 aa）	Not recorded	SEP PHOSPHOSERINE × 1	X-RAY DIFFRACTION X-ray crystallization conditions VAPOR DIFFUSION, HANGING DROP；293 K；2:1 drop ratio 0.1 M CHES (pH 7.4) 0.1M magnesium acetate 10% ethanol (v/v) Crystal soaked with 5 mM OPLS	Resolution 1.30 Å R-free 0.156
8G6I Coagulation factor VIII bound to a patient-derived anti-C1 domain antibody inhibitor Deposited 2023-02-15	Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Insufficient information Heteromer；Protein × 3 PDB declaration: trimeric	Chain A 1–405（405 aa） Chain A 1438–1820（383 aa）	Not recorded	No recorded non-water small molecule	ELECTRON MICROSCOPY cryo-EM buffer pH 7.4；150 mM NaCl, 20 mM HEPES pH 7.4 cryo-EM vitrification conditions Cryogen ETHANE	Resolution 4.23 Å
8TY1 Cryo-EM structure of coagulation factor VIII bound to NB2E9 Deposited 2023-08-24	Different construct Different oligomeric state Different ligand/ion Different experimental method Different experimental conditions Different structure-quality metrics	Assembly 1 Insufficient information Heteromer；Protein × 3 PDB declaration: trimeric	Chain A 1–391（391 aa） Chain A 1438–1820（383 aa）	Not recorded	No recorded non-water small molecule	ELECTRON MICROSCOPY cryo-EM buffer pH 7.4；20 mM HEPES (pH 7.4) and 150 mM NaCl cryo-EM vitrification conditions Cryogen ETHANE	Resolution 3.46 Å

6 other PDB entries and 7 assemblies. Open the comparison page and filter oligomeric states

View Construct and Data Evidence

UniProt name	FA8_PIG
Isoform	—
PDB entities	1
Chains and sequence ranges	Author chain A； PDBConstruct 1–405； UniProt 1–405 Author chain A； PDBConstruct 772–1154； UniProt 1438–1820

The page prioritizes protein identity, the current assembly, associated components, oligomeric state and cross-PDB links. Chain mapping and sequence ranges are retained as data evidence. Internal IDs, import timestamps and assembly operation expressions are maintenance fields and are not shown here.

SAXS scattering curve SAXS Profile

P(r) Distance Distribution P(r) Distribution

Download Download SAXS (.dat) P(r) (.pr.out) CIF

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2. Structure Basics 2. Structure Basics

Entry ID entry_id	7k66
Deposition date deposition_date	2020-09-18
Structure title title	Structure of Blood Coagulation Factor VIII in Complex with an Anti-C1 Domain Pathogenic Antibody Inhibitor
Keywords keywords	Antibody, inhibitor, BLOOD CLOTTING, BLOOD CLOTTING-Immune System complex; BLOOD CLOTTING/Immune System
Experimental Method method	X-RAY DIFFRACTION

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3. SAXS Parameters (CRYSOL theoretical calculation) 3. SAXS Parameters (CRYSOL)

Radius of gyration Rg (Guinier) rg_guinier	44.76 Å
Radius of gyration Rg (electron density) rg_electron	44.82 Å
Forward intensity I(0) i0	543141000.00
Molecular weight molecular_weight	193060.0 kDa
Excluded volume excluded_volume	241810 Å³
Envelope volume envelope_volume	334530 Å³
Hydration-shell volume shell_volume	64543 Å³
Envelope diameter envelope_diameter	162.9 Å
Shell Rg shell_rg	47.17 Å
Envelope Rg envelope_rg	45.34 Å
Shape Rg shape_rg	44.79 Å
Total Rg total_rg	45.02 Å
Total atoms total_atoms	13605
Residues n_residues	1695
Spherical-harmonic order n_harmonics	20
q range q_range	— – 0.5000 Å⁻¹
Data points n_points	101
Shell type shell_type	directional
Solvent electron density solvent_density	0.3340 e/Å³
Shell contrast contrast_shell	0.0300 e/Å³
CRYSOL version crysol_version	4.1.3

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4. P(r) Distance Distribution (GNOM inversion) 4. P(r) Analysis (GNOM)

Maximum dimension Dmax dmax	153.4 Å
Rg (real space) rg_real	45.07 Å
Rg uncertainty (real space) rg_real_error	1.32 Å
I(0) (real space) i0_real	5.4310e+08
I(0) uncertainty (real space) i0_real_error	1.0600e+07
Rg (reciprocal space) rg_reciprocal	44.77 Å
I(0) (reciprocal space) i0_reciprocal	542900000.0000
Solution quality estimate total_estimate	0.8324
Solution quality rating solution_quality	GOOD a GOOD solution
P(r) peaks n_peaks	1
Primary peak position r_peak_primary	43.8 Å
Skewness Skewness skewness	0.523
Kurtosis Kurtosis kurtosis	-0.247
Angular range angular_range	— – 0.1750 Å⁻¹
Current regularization parameter α current_alpha	0.0002
Highest regularization parameter α highest_alpha	80750000.0000
Real-space data points n_real_points	36
GNOM version gnom_version	4.1.3
Quality Criteria quality_criteria	AN1: 0.000; Oscil: 0.794; Stabil: 1.000; Sysdev: 1.000; Positv: 1.000; Valcen: 0.883; Smooth: 0.553

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5. Crystallography and Experiment 5. Crystallography & Experiment

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6. Entities and Polymers Entities & Polymers (10)

Entity 1 Entity 1 polymer

Description description	Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII
Molecular weight formula_weight	168287.0 kDa
Source method src_method	man
Copy count entity_count	1

Polymer Polymer

Polymer type type	polypeptide(L)
Chain IDs strand_id	A
Atom count atom_count	13606

MQLELSTCVFLCLLPLGFSAIRRYYLGAVELSWDYRQSELLRELHVDTRFPATAPGALPLGPSVLYKKTVFVEFTDQLFSVARPRPPWMGLLGPTIQAEVYDTVVVTLKNMASHPVSLHAVGVSFWKSSEGAEYEDHTSQREKEDDKVLPGKSQTYVWQVLKENGPTASDPPCLTYSYLSHVDLVKDLNSGLIGALLVCREGSLTRERTQNLHEFVLLFAVFDEGKSWHSARNDSWTRAMDPAPARAQPAMHTVNGYVNRSLPGLIGCHKKSVYWHVIGMGTSPEVHSIFLEGHTFLVRHHRQASLEISPLTFLTAQTFLMDLGQFLLFCHISSHHHGGMEAHVRVESCAEEPQLRRKADEEEDYDDNLYDSDMDVVRLDGDDVSPFIQIRSVAKKHPKTWVHYIAAEEEDWDYAPLVLAPDDRSYKSQYLNNGPQRIGRKYKKVRFMAYTDETFKTREAIQHESGILGPLLYGEVGDTLLIIFKNQASRPYNIYPHGITDVRPLYSRRLPKGVKHLKDFPILPGEIFKYKWTVTVEDGPTKSDPRCLTRYYSSFVNMERDLASGLIGPLLICYKESVDQRGNQIMSDKRNVILFSVFDENRSWYLTENIQRFLPNPAGVQLEDPEFQASNIMHSINGYVFDSLQLSVCLHEVAYWYILSIGAQTDFLSVFFSGYTFKHKMVYEDTLTLFPFSGETVFMSMENPGLWILGCHNSDFRNRGMTALLKVSSCDKNTGDYYEDSYEDISAYLLSKNNAIEPRSFAQNSRPPSASAPKPPVLRRHQRDISLPTFQPEEDKMDYDDIFSTETKGEDFDIYGEDENQDPRSFQKRTRHYFIAAVEQLWDYGMSESPRALRNRAQNGEVPRFKKVVFREFADGSFTQPSYRGELNKHLGLLGPYIRAEVEDNIMVTFKNQASRPYSFYSSLISYPDDQEQGAEPRHNFVQPNETRTYFWKVQHHMAPTEDEFDCKAWAYFSDVDLEKDVHSGLIGPLLICRANTLNAAHGRQVTVQEFALFFTIFDETKSWYFTENVERNCRAPCHLQMEDPTLKENYRFHAINGYVMDTLPGLVMAQNQRIRWYLLSMGSNENIHSIHFSGHVFSVRKKEEYKMAVYNLYPGVFETVEMLPSKVGIWRIECLIGEHLQAGMSTTFLVYSKKCQTPLGMASGHIRDFQITASGQYGQWAPKLARLHYSGSINAWSTKEPFSWIKVDLLAPMIIHGIKTQGARQKFSSLYISQFIIMYSLDGKKWQTYRGNSTGTLMVFFGNVDSSGIKHNIFNPPIIARYIRLHPTHYSIRSTLRMELMGCDLNSCSMPLGMESKAISDAQITASSYFTNMFATWSPSKARLHLQGRSNAWRPQVNNPKEWLQVDFQKTMKVTGVTTQGVKSLLTSMYVKEFLISSSQDGHQWTLFFQNGKVKVFQGNQDSFTPVVNSLDPPLLTRYLRIHPQSWVHQIALRMEVLGCEAQDLY

来源 Source

来源生物 organism	Homo sapiens
通用名 common_name	Human
NCBI Class编号 ncbi_taxonomy_id	9606
宿主生物 host_organism	Cricetulus griseus

Entity 2 Entity 2 polymer

Description description	2A9 heavy chain
Molecular weight formula_weight	23979.8 kDa
Source method src_method	man
Copy count entity_count	1

Polymer Polymer

Polymer type type	polypeptide(L)
Chain IDs strand_id	B

QIQLVQSGPELKKPGETVKISCKASGYTFTDYSMHWVKQAPGKGLKWMGWINTETGEPTYADDFKGRFAFSLETSASSAYLQINNLKNEDTATYFCARCGNYVDYAIDYWGQGTSVTVSSAKTTAPSVYPLAPVCGDTTGSSVTLGCLVKGYFPEPVTLTWNSGSLSSGVHTFPAVLQSDLYTLSSSVTVTSSTWPSQSITCNVAHPASSTKVDKKIEPRGPA

来源 Source

来源生物 organism	Mus musculus
NCBI Class编号 ncbi_taxonomy_id	10090
宿主生物 host_organism	Mus musculus

Entity 3 Entity 3 polymer

Description description	2A9 light chain
Molecular weight formula_weight	23297.7 kDa
Source method src_method	man
Copy count entity_count	1

Polymer Polymer

Polymer type type	polypeptide(L)
Chain IDs strand_id	C

ENVLTQSPAIMSASLGEKVTMSCRATSSVNYMYWYQQKSDASPKLWIFFTSSLAPGVPARFSGSGSGNSYSLTISSVEGEAAATYYCQQFTSSPFGSGTKLEIKAKRADAAPTVSIFPPSSEQLTSGGASVVCFLNNFYPKDINVKWKIDGSERQNGVLNSWTDQDSKDSTYSMSSTLTLTKDEYERHNSYTCEATHSTKTSPIVKSFNRNEC

来源 Source

来源生物 organism	Mus musculus
NCBI Class编号 ncbi_taxonomy_id	10090
宿主生物 host_organism	Mus musculus

Entity 4 Entity 4 branched

Description description	;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 ;
Molecular weight formula_weight	910.8 kDa
Source method src_method	man
Copy count entity_count	1

Entity 5 Entity 5 branched

Description description	2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose
Molecular weight formula_weight	424.4 kDa
Source method src_method	man
Copy count entity_count	1

Entity 6 Entity 6 non-polymer

Description description	2-acetamido-2-deoxy-beta-D-glucopyranose
Molecular weight formula_weight	221.2 kDa
Source method src_method	syn
Copy count entity_count	1

Entity 7 Entity 7 non-polymer

Description description	COPPER (II) ION
Molecular weight formula_weight	63.5 kDa
Source method src_method	syn
Copy count entity_count	1

Entity 8 Entity 8 non-polymer

Description description	CALCIUM ION
Molecular weight formula_weight	40.1 kDa
Source method src_method	syn
Copy count entity_count	1

Entity 9 Entity 9 non-polymer

Description description	ZINC ION
Molecular weight formula_weight	65.4 kDa
Source method src_method	syn
Copy count entity_count	1

Entity 10 Entity 10 water

Description description	water
Molecular weight formula_weight	18.0 kDa
Source method src_method	nat
Copy count entity_count	1

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7. Fold Classification (SCOP + CATH) 2 domains

CATH v4.4 (2 domains)

Domain ID domain_id	7k66C01
Class class	2 — Mainly Beta
Architecture architecture	60 — Sandwich
Topology topology	40 — Immunoglobulin-like
Homologous superfamily homologous superfamily	10 — Immunoglobulins

Domain ID domain_id	7k66C02
Class class	2 — Mainly Beta
Architecture architecture	60 — Sandwich
Topology topology	40 — Immunoglobulin-like
Homologous superfamily homologous superfamily	10 — Immunoglobulins

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8. Citations (1)

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Unit-cell length a length_a	116.721 Å
Unit-cell length b length_b	116.721 Å
Unit-cell length c length_c	371.057 Å
Unit-cell angle α angle_alpha	90.00 °
Unit-cell angle β angle_beta	90.00 °
Unit-cell angle γ angle_gamma	90.00 °
Molecules per unit cell Z z_pdb	8

Space group space_group	P 41 21 2
Space-group number int_tables_number	92
Crystal system crystal_system	tetragonal

Resolution resolution	3.920 Å
Low-resolution limit resolution_low	49.490 Å
R factor r_factor	0.2341
R_work r_work	0.2257
R_free r_free	0.3219

fit	/app/data/saxs_computation/k6/7k66/7k66.fit
pddf	/app/data/pr_computation/k6/7k66/7k66.pr.out
profile	/app/data/saxs_computation/k6/7k66/7k66.dat

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

1. Protein Identity and Related Structures Protein Identity & Related Structures

Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII

State in the Current Structure

Other States of the Same Protein in the Database

Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII,Coagulation factor VIII

State in the Current Structure

Other States of the Same Protein in the Database

SAXS scattering curve SAXS Profile

P(r) Distance Distribution P(r) Distribution

2. Structure Basics 2. Structure Basics

3. SAXS Parameters (CRYSOL theoretical calculation) 3. SAXS Parameters (CRYSOL)

4. P(r) Distance Distribution (GNOM inversion) 4. P(r) Analysis (GNOM)

5. Crystallography and Experiment 5. Crystallography & Experiment

Unit cell Unit Cell

Symmetry Symmetry

Refinement Refinement

Diffraction experiment Diffraction

6. Entities and Polymers Entities & Polymers (10)

Entity 1 Entity 1 polymer

Polymer Polymer

来源 Source

Entity 2 Entity 2 polymer

Polymer Polymer

来源 Source

Entity 3 Entity 3 polymer

Polymer Polymer

来源 Source

Entity 4 Entity 4 branched

Entity 5 Entity 5 branched

Entity 6 Entity 6 non-polymer

Entity 7 Entity 7 non-polymer

Entity 8 Entity 8 non-polymer

Entity 9 Entity 9 non-polymer

Entity 10 Entity 10 water

7. Fold Classification (SCOP + CATH) 2 domains

CATH v4.4 (2 domains)

8. Citations (1)

9. Files and Curves (10)

Files (7)

Curves (3)