PiSITE

PDB ID	2A73	CHAIN	B
Protein name	Complement C3
Uniprot Accession	P01024
The number of similar proteins	25
The number of binding states	12
The number of binding partners	13

Binding state	Binding partners
	2A73 (CHAIN: B)
1	P01024
2	P01024 6RU5
3	P01024 2WII
4	P01024 Q9Y279
5	P01024 P00751
6	P01024 P15529
7	P01024 3G6J 3G6J
8	P01024 P00751 P27918
9	P01024 P08603 P05156 P05156
10	P01024 P01024 P01024 P11215
11	P01024 2WIN 2WIN P00751 P00751
12	P01024 P01024 P01024 P11215 P11215

Molecule viewer

Only interaction residues

#binding
partners

	>9
	8
	7
	6
	5
	4
	3
	2
	1
	0

Sequence information

1

VQLTEKRMDK

VGKYPKELRK

CCEDGMRENP

MRFSCQRRTR

FISLGEACKK

50

51

VFLDCCNYIT

ELRRQHARAS

HLGLARSNLD

EDIIAEENIV

SRSEFPESWL

100

101

WNVEDLKEPP

KNGISTKLMN

IFLKDSITTW

EILAVSMSDK

KGICVADPFE

150

151

VTVMQDFFID

LRLPYSVVRN

EQVEIRAVLY

NYRQNQELKV

RVELLHNPAF

200

201

CSLATTKRRH

QQTVTIPPKS

SLSVPYVIVP

LKTGLQEVEV

KAAVYHHFIS

250

251

DGVRKSLKVV

PEGIRMNKTV

AVRTLDPERL

GREGVQKEDI

PPADLSDQVP

300

301

DTESETRILL

QGTPVAQMTE

DAVDAERLKH

LIVTPSGCGE

QNMIGMTPTV

350

351

IAVHYLDETE

QWEKFGLEKR

QGALELIKKG

YTQQLAFRQP

SSAFAAFVKR

400

401

APSTWLTAYV

VKVFSLAVNL

IAIDSQVLCG

AVKWLILEKQ

KPDGVFQEDA

450

451

PVIHQEMIGG

LRNNNEKDMA

LTAFVLISLQ

EAKDICEEQV

NSLPGSITKA

500

501

GDFLEANYMN

LQRSYTVAIA

GYALAQMGRL

KGPLLNKFLT

TAKDKNRWED

550

551

PGKQLYNVEA

TSYALLALLQ

LKDFDFVPPV

VRWLNEQRYY

GGGYGSTQAT

600

601

FMVFQALAQY

QKDAPDHQEL

NLDVSLQLPS

RSSKITHRIH

WESASLLRSE

650

651

ETKENEGFTV

TAEGKGQGTL

SVVTMYHAKA

KDQLTCNKFD

LKVTIKPAPE

700

701

TEKRPQDAKN

TMILEICTRY

RGDQDATMSI

LDISMMTGFA

PDTDDLKQLA

750

751

NGVDRYISKY

ELDKAFSDRN

TLIIYLDKVS

HSEDDCLAFK

VHQYFNVELI

800

801

QPGAVKVYAY

YNLEESCTRF

YHPEKEDGKL

NKLCRDELCR

CAEENCFIQK

850

851

SDDKVTLEER

LDKACEPGVD

YVYKTRLVKV

QLSNDFDEYI

MAIEQTIKSG

900

901

SDEVQVGQQR

TFISPIKCRE

ALKLEEKKHY

LMWGLSSDFW

GEKPNLSYII

950

951

GKDTWVEHWP

EEDECQDEEN

QKQCQDLGAF

TESMVVFGCP

N

1000

Binding partner information

P01024

Complement C3

Binding partner information

	P01024	Complement C3
	6RU5	hC3Nb1

Binding partner information

	P01024	COMPLEMENT C3 BETA CHAIN
	2WII	COMPLEMENT FACTOR H

Binding partner information

	P01024	Complement C3 beta chain
	Q9Y279	V-set and immunoglobulin domain-containing protein 4

Binding partner information

	P01024	COMPLEMENT C3 BETA CHAIN
	P00751	COMPLEMENT FACTOR B

Binding partner information

	P01024	COMPLEMENT C3
	P15529	MEMBRANE COFACTOR PROTEIN

Binding partner information

	P01024	Complement C3 beta chain
	3G6J	Fab heavy chain
	3G6J	Fab light chain

Binding partner information

	P01024	Complement C3 beta chain
	P00751	Complement factor B
	P27918	Properdin

Binding partner information

	P01024	Complement C3
	P08603	Complement factor H,Complement factor H
	P05156	Complement factor I
	P05156	Complement factor I

Binding partner information

	P01024	Complement C3b alpha' chain
	P01024	Complement C3 beta chain
	P01024	Complement C3 beta chain
	P11215	Integrin alpha-M

Binding partner information

	P01024	COMPLEMENT C3 BETA CHAIN
	2WIN	STAPHYLOCOCCAL COMPLEMENT INHIBITOR
	2WIN	STAPHYLOCOCCAL COMPLEMENT INHIBITOR
	P00751	COMPLEMENT FACTOR B
	P00751	COMPLEMENT FACTOR B

Binding partner information

	P01024	Complement C3b alpha' chain
	P01024	Complement C3 beta chain
	P01024	Complement C3 beta chain
	P11215	Integrin alpha-M
	P11215	Integrin alpha-M

Variants

Residue	AA	Source / dbSNP	Clinical Significance	Allele Frequency (> 0.0001)	AlphaMissense pathogenicity	Disease name
651_VAL	MET	8.3kJPN chr19:6707507 rs534845907	-	0.0001	0.2306	-
665_PRO	THR	8.3kJPN chr19:6707273 -	-	0.0001	0.0796	-
690_ARG	HIS	8.3kJPN chr19:6707197 rs773113880	-	0.0005	0.0969	-
690_ARG	CYS	8.3kJPN chr19:6707198 rs763061857	-	0.0001	0.3711	-
713_ARG	TRP	VAR_063215 rs117793540	LP/P	-	0.3176	Hemolytic uremic syndrome atypical 5 (AHUS5) [MIM:612925]
713_ARG	TRP	gnomAD chr19:6707129 rs117793540	-	0.00218304	0.3176	-
713_ARG	TRP	8.3kJPN chr19:6707129 rs117793540	-	0.0001	0.3176	-
714_ARG	GLN	8.3kJPN chr19:6707125 rs578116271	-	0.0005	0.0721	-
715_GLN	TER	ClinVar chr19:6707123 -	Pathogenic	-	-	not provided [MedGen:C3661900]
718_ARG	TRP	8.3kJPN chr19:6707114 rs945676974	-	0.0001	0.2047	-
742_ARG	TER	ClinVar chr19:6702546 -	Pathogenic	-	-	not provided [MedGen:C3661900]
743_SER	THR	8.3kJPN chr19:6702542 -	-	0.0005	0.0781	-
753_VAL	ALA	8.3kJPN chr19:6702512 -	-	0.0001	0.1083	-
793_ILE	MET	8.3kJPN chr19:6697801 -	-	0.0001	0.1859	-
800_GLU	LYS	8.3kJPN chr19:6697782 -	-	0.0001	0.3137	-
805_GLN	TER	ClinVar chr19:6697767 -	Pathogenic	-	-	not provided [MedGen:C3661900]
807_PHE	LEU	8.3kJPN chr19:6697759 -	-	0.0001	0.9837	-
822_GLN	ARG	ClinVar chr19:6697715 rs1599510478	Likely pathogenic	-	0.6996	Atypical hemolytic-uremic syndrome with C3 anomaly [MONDO:MONDO:0013043,MedGen:C2752037,OMIM:612925,Orphanet:2134]
841_ARG	LYS	VAR_019206 rs11569472	LB/B	-	0.1584	-
849_ALA	GLY	8.3kJPN chr19:6697539 -	-	0.0001	0.184	-
864_VAL	ILE	8.3kJPN chr19:6697495 rs149395057	-	0.0003	0.0643	-
868_PRO	ARG	8.3kJPN chr19:6697482 rs753876258	-	0.0001	0.1729	-
877_VAL	ILE	8.3kJPN chr19:6697456 -	-	0.0001	0.076	-
878_ILE	MET	8.3kJPN chr19:6697451 rs763155610	-	0.0021	0.1852	-
890_VAL	LEU	8.3kJPN chr19:6697417 -	-	0.0001	0.7684	-
908_LYS	ASN	8.3kJPN chr19:6697361 -	-	0.0001	0.5224	-
967_GLN	TER	ClinVar chr19:6694631 -	Pathogenic	-	-	not provided [MedGen:C3661900]
991_GLN	HIS	8.3kJPN chr19:6694557 -	-	0.0001	0.9919	-
995_GLY	CYS	8.3kJPN chr19:6694547 -	-	0.0003	0.4243	-
1007_ASP	ASN	ClinVar chr19:6694511 rs1599507415	Likely pathogenic	-	0.5718	Mesangiocapillary glomerulonephritis [Human Phenotype Ontology:HP:0000793,MONDO:MONDO:0002461,MedGen:C0017662]
1020_ARG	LEU	VAR_063655 -	LP/P	-	0.8973	Hemolytic uremic syndrome atypical 5 (AHUS5) [MIM:612925]
1072_ALA	VAL	VAR_063216 rs121909584	LP/P	-	0.1579	Hemolytic uremic syndrome atypical 5 (AHUS5) [MIM:612925]
1079_CYS	TRP	ClinVar chr19:6693022 rs1236557685	Likely pathogenic	-	0.9877	not provided [MedGen:C3661900]
1093_ASP	ASN	VAR_063217 rs121909585	LP/P	-	0.1388	Hemolytic uremic syndrome atypical 5 (AHUS5) [MIM:612925]
1122_THR	ALA	8.3kJPN chr19:6690699 -	-	0.0001	0.849	-
1135_ILE	THR	ClinVar chr19:6690659 rs1918142335	Pathogenic	-	0.2583	Atypical hemolytic-uremic syndrome [MONDO:MONDO:0016244,MedGen:C2931788,Orphanet:2134]
1136_CYS	TRP	VAR_063218 -	LP/P	-	0.9941	Hemolytic uremic syndrome atypical 5 (AHUS5) [MIM:612925]
1139_GLN	LYS	VAR_063219 -	LP/P	-	0.0872	Hemolytic uremic syndrome atypical 5 (AHUS5) [MIM:612925]
1202_GLY	ASP	VAR_019207 rs11569534	LB/B	-	0.1152	-
1202_GLY	ASP	gnomAD chr19:6686274 rs11569534	-	0.00147915	0.1152	-
1230_VAL	ILE	gnomAD chr19:6686191 rs537300095	-	0.000258453	0.0874	-
1230_VAL	ILE	8.3kJPN chr19:6686191 rs537300095	-	0.0001	0.0874	-
1241_GLY	ALA	8.3kJPN chr19:6686157 -	-	0.0001	0.3941	-
1249_ALA	SER	8.3kJPN chr19:6685157 -	-	0.0001	0.1662	-
1264_ALA	THR	8.3kJPN chr19:6685112 rs556995881	-	0.0001	0.0857	-
1281_ARG	HIS	ClinVar chr19:6685060 rs775015499	Likely pathogenic	-	0.1238	Atypical hemolytic-uremic syndrome with C3 anomaly [MONDO:MONDO:0013043,MedGen:C2752037,OMIM:612925,Orphanet:2134]
1281_ARG	CYS	8.3kJPN chr19:6685061 rs567359713	-	0.0001	0.4345	-
1286_THR	ILE	8.3kJPN chr19:6685045 -	-	0.0001	0.0766	-
1345_ILE	THR	VAR_019208 rs11569541	LB/B	-	0.4659	-
1345_ILE	THR	gnomAD chr19:6684591 rs11569541	-	0.000628241	0.4659	-
1359_LYS	ARG	8.3kJPN chr19:6684429 rs1232402436	-	0.0001	0.0787	-
1361_THR	ASN	gnomAD chr19:6684423 rs139100972	-	0.000151096	0.3931	-
1371_ARG	GLN	8.3kJPN chr19:6682235 rs575119407	-	0.0005	0.082	-
1371_ARG	TRP	8.3kJPN chr19:6682236 rs148227405	-	0.0001	0.1423	-
1372_GLY	ARG	8.3kJPN chr19:6682233 rs200597513	-	0.0001	0.5419	-
1378_MET	VAL	8.3kJPN chr19:6682215 -	-	0.0001	0.8223	-
1418_ASP	ALA	gnomAD chr19:6681983 rs147116781	-	0.000258453	0.1069	-
1418_ASP	ASN	8.3kJPN chr19:6681984 rs772034590	-	0.0001	0.0633	-
1435_ASP	HIS	gnomAD chr19:6680256 rs113742728	-	0.000278352	0.4283	-
1442_HIS	ASP	VAR_063220 -	LP/P	-	0.8904	Hemolytic uremic syndrome atypical 5 (AHUS5) [MIM:612925]
1459_ALA	THR	8.3kJPN chr19:6680184 rs1293706769	-	0.0001	0.1674	-
1462_ASN	SER	8.3kJPN chr19:6680174 -	-	0.0001	0.1103	-
1473_PRO	ALA	8.3kJPN chr19:6679481 -	-	0.0002	0.1203	-
1490_ARG	CYS	ClinVar chr19:6679430 rs1408701335	Likely pathogenic	-	0.5636	Atypical hemolytic-uremic syndrome with C3 anomaly [MONDO:MONDO:0013043,MedGen:C2752037,OMIM:612925,Orphanet:2134]
1490_ARG	HIS	gnomAD chr19:6679429 rs142868256	-	0.000111343	0.1193	-
1499_GLN	ARG	VAR_029792 rs7256789	LB/B	-	0.0733	-
1527_LEU	MET	gnomAD chr19:6678452 rs149202905	-	0.000981862	0.1966	-
1579_HIS	ASN	VAR_029793 rs1803225	LB/B	-	0.0781	-
1582_MET	THR	8.3kJPN chr19:6678202 rs761512303	-	0.0004	0.5505	-
1597_SER	ARG	VAR_029326 rs2230210	LB/B	-	0.236	-
1597_SER	ARG	gnomAD chr19:6678030 rs2230210	-	0.00118499	0.236	-
1602_LYS	TER	ClinVar chr19:6678015 -	Likely pathogenic	-	-	not provided [MedGen:C3661900]
1604_THR	SER	8.3kJPN chr19:6678008 rs1386293935	-	0.0001	0.2983	-
1607_GLU	ASP	8.3kJPN chr19:6677998 rs200816772	-	0.0001	0.8922	-

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Variants

Reference