PiSITE

PDB ID	1DM4	CHAIN	B
Protein name	PROTEIN (MUTANT ALPHA THROMBIN:HEAVY CHAIN)
Uniprot Accession	P00734
The number of similar proteins	455
The number of binding states	29
The number of binding partners	22

Binding state	Binding partners
	1DM4 (CHAIN: B)
1	P00734
2	Monomeric state
3	P00734
4	Q846V4
5	P00734
6	P07359
7	P01050
8	P00734 P05546
9	P00734 P01008
10	P00734 P00734
11	P00734 P05154
12	P00734 P07204
13	P00734 P07359
14	P00735 P00734
15	P00734 P85800
16	P00734 P12259
17	P00734 Q86FP9
18	P00734 Q9NJS1
19	P00734 P09945
20	P00734 5E8E
21	P00734 O97373
22	P00734 A2IAB2
23	P00734 P00974
24	P00734 P00734 P01008
25	P00734 P00734 P00734
26	P00734 P00734 Q25163
27	P00734 P02679 P02675 P02671
28	P00734 P00734 Q25163 Q25163
29	P00734 P00734 P00734 P00734 Q25163

Molecule viewer

Only interaction residues

#binding
partners

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Sequence information

1

RIVEGSDAEI

GMSPWQVMLF

RKSPQELLCG

ASLISDRWVL

TAAHCLLYPP

50

51

WDKNFTENDL

LVRIGKHSRT

RYERNIEKIS

MLEKIYIHPR

YNWRENLDRD

100

101

IALMKLKKPV

AFSDYIHPVC

LPDRETAASL

LQAGYKGRVT

GWGNLKETWT

150

151

ANVGKGQPSV

LQVVNLPIVE

RPVCKDSTRI

RITDNMFCAG

YKPDEGKRGD

200

201

ACEGDAGGPF

VMKSPFNNRW

YQMGIVSWGE

GCDRDGKYGF

YTHVFRLKKW

250

251

IQKVIDQFGE

300

Binding partner information

P00734

PROTEIN (ALPHA THROMBIN:LIGHT CHAIN)

Binding partner information

P00734

Prothrombin

Binding partner information

Q846V4

Staphylocoagulase

Binding partner information

P00734

ALPHA-THROMBIN (LARGE SUBUNIT)

Binding partner information

P07359

Platelet glycoprotein Ib alpha chain

Binding partner information

P01050

Hirudin variant-1

Binding partner information

	P00734	Thrombin, light chain
	P05546	HEPARIN COFACTOR II

Binding partner information

	P00734	thrombin
	P01008	Antithrombin-III

Binding partner information

	P00734	Thrombin
	P00734	Thrombin

Binding partner information

	P00734	Prothrombin
	P05154	Plasma serine protease inhibitor

Binding partner information

	P00734	Prothrombin
	P07204	Thrombomodulin

Binding partner information

	P00734	Human Alpha Thrombin
	P07359	Platelet glycoprotein Ib alpha chain precursor

Binding partner information

	P00735	Prothrombin
	P00734	ALPHA-THROMBIN LIGHT CHAIN

Binding partner information

	P00734	Thrombin light chain
	P85800	Variegin

Binding partner information

	P00734	Thrombin light chain
	P12259	Coagulation factor V

Binding partner information

	P00734	THROMBIN LIGHT CHAIN
	Q86FP9	THROMBIN INHIBITOR MADANIN 1

Binding partner information

	P00734	Thrombin
	Q9NJS1	Salivary anti-thrombin peptide anophelin

Binding partner information

	P00734	ALPHA-THROMBIN (SMALL SUBUNIT)
	P09945	HIRUDIN VARIANT 2

Binding partner information

	P00734	THROMBIN LIGHT CHAIN
	5E8E	IGA FAB HEAVY CHAIN

Binding partner information

	P00734	Prothrombin
	O97373	Tsetse thrombin inhibitor

Binding partner information

	P00734	Thrombin light chain
	A2IAB2	Putative secreted salivary protein

Binding partner information

	P00734	THROMBIN
	P00974	BOVINE PANCREATIC TRYPSIN INHIBITOR

Binding partner information

	P00734	Thrombin
	P00734	Thrombin
	P01008	Antithrombin-III

Binding partner information

	P00734	Thrombin heavy chain
	P00734	Thrombin heavy chain
	P00734	Thrombin light chain

Binding partner information

	P00734	THROMBIN
	P00734	THROMBIN
	Q25163	HAEMADIN

Binding partner information

	P00734	Thrombin light chain
	P02679	Fibrinogen gamma chain
	P02675	Fibrinogen beta chain
	P02671	Fibrinogen alpha chain

Binding partner information

	P00734	THROMBIN
	P00734	THROMBIN
	Q25163	HAEMADIN
	Q25163	HAEMADIN

Binding partner information

	P00734	THROMBIN
	P00734	THROMBIN
	P00734	THROMBIN
	P00734	THROMBIN
	Q25163	HAEMADIN

Variants

Residue	AA	Source / dbSNP	Clinical Significance	Allele Frequency (> 0.0001)	AlphaMissense pathogenicity	Disease name
17_VAL	GLU	ClinVar chr11:46748351 rs747234596	Likely pathogenic	-	0.9231	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
32_MET	THR	VAR_006714 rs121918481	LP/P	-	0.9427	Factor II deficiency (FA2D) [MIM:613679]
32_MET	THR	ClinVar chr11:46749554 rs121918481	Pathogenic	-	0.9427	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
35_ARG	TRP	8.3kJPN chr11:46749562 rs147456134	-	0.0001	0.3319	-
37_PRO	THR	VAR_011782 rs5897	LB/B	-	0.6564	-
38_GLN	TER	ClinVar chr11:46749574 rs2064886777	Pathogenic/Likely pathogenic	-	-	not provided\|Congenital prothrombin deficiency [MedGen:C3661900\|MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
49_ASP	GLU	8.3kJPN chr11:46749609 rs1255378194	-	0.0002	0.4807	-
50_ARG	HIS	8.3kJPN chr11:46749611 rs765024112	-	0.0001	0.1029	-
52_VAL	ILE	8.3kJPN chr11:46749616 -	-	0.0002	0.0714	-
63_ASP	GLU	8.3kJPN chr11:46749678 -	-	0.0001	0.3151	-
66_VAL	MET	ClinVar chr11:46749685 rs1310397756	Likely pathogenic	-	0.4919	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
67_ARG	CYS	VAR_006715 rs121918479	LP/P	-	0.8904	Factor II deficiency (FA2D) [MIM:613679]
67_ARG	CYS	ClinVar chr11:46749688 rs121918479	Pathogenic	-	0.8904	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
67_ARG	HIS	ClinVar chr11:46749689 rs121918485	Pathogenic	-	0.7753	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
67_ARG	CYS	8.3kJPN chr11:46749688 rs121918479	-	0.0001	0.8904	-
73_ARG	HIS	VAR_006716 rs121918482	LP/P	-	0.6068	Factor II deficiency (FA2D) [MIM:613679]
73_ARG	HIS	ClinVar chr11:46749707 rs121918482	Pathogenic	-	0.6068	DYSPROTHROMBINEMIA PROTHROMBIN HIMI-II [-]
77A_ARG	TER	8.3kJPN chr11:46750221 -	-	0.0001	-	-
97_ARG	GLN	8.3kJPN chr11:46750282 rs143498218	-	0.0004	0.0973	-
101_ARG	TRP	VAR_006717 rs121918478	LP/P	-	0.5518	Factor II deficiency (FA2D) [MIM:613679]
101_ARG	TRP	ClinVar chr11:46750296 rs121918478	Pathogenic	-	0.5518	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
111_PRO	ALA	8.3kJPN chr11:46750326 rs377462682	-	0.0002	0.1049	-
125_ASP	GLY	8.3kJPN chr11:46750369 -	-	0.0001	0.1959	-
136_GLY	GLU	ClinVar chr11:46750953 rs2134537035	Likely pathogenic	-	0.9911	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
137_ARG	GLN	ClinVar chr11:46750956 rs202003146	Pathogenic	-	0.4352	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
146_GLU	ALA	VAR_006718 -	LP/P	-	0.563	Factor II deficiency (FA2D) [MIM:613679]
164_GLU	GLN	VAR_068913 -	LB/B	-	0.1123	-
167_VAL	ILE	8.3kJPN chr11:46751060 rs768032315	-	0.0008	0.075	-
175_ARG	LEU	gnomAD chr11:46751085 rs143064939	-	0.0048737	0.1903	-
187_ARG	TER	ClinVar chr11:46760621 -	Pathogenic	-	-	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
207_TRP	TER	ClinVar chr11:46760834 -	Likely pathogenic	-	-	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
221_ASP	GLU	ClinVar chr11:46760874 rs121918486	Pathogenic	-	0.8671	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
221A_ARG	LEU	ClinVar chr11:46760876 rs387907201	Pathogenic	-	0.6818	Thrombophilia due to thrombin defect [MONDO:MONDO:0008559,MedGen:C3160733,OMIM:188050]
221A_ARG	GLN	ClinVar chr11:46760876 rs387907201	Pathogenic	-	0.2416	Thrombophilia due to thrombin defect\|not provided\|Congenital prothrombin deficiency [MONDO:MONDO:0008559,MedGen:C3160733,OMIM:188050\|MedGen:C3661900\|MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
221A_ARG	TRP	ClinVar chr11:46760875 -	Pathogenic	-	0.5224	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
226_GLY	VAL	VAR_006719 rs121918480	LP/P	-	0.9835	Factor II deficiency (FA2D) [MIM:613679]
226_GLY	VAL	ClinVar chr11:46760891 rs121918480	Pathogenic	-	0.9835	Congenital prothrombin deficiency [MONDO:MONDO:0013361,MedGen:C0272317,OMIM:613679,Orphanet:325]
230_HIS	TYR	8.3kJPN chr11:46760902 -	-	0.0001	0.4482	-
233_ARG	HIS	8.3kJPN chr11:46760912 rs758608905	-	0.0001	0.407	-
234_LEU	PRO	ClinVar chr11:46760915 rs1592422740	Likely pathogenic	-	0.9898	Prolonged prothrombin time [Human Phenotype Ontology:HP:0008151,MedGen:C0853225]

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Variants

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