PiSITE

PDB ID	3PRX	CHAIN	A
Protein name	Complement C5
Uniprot Accession	P01031
The number of similar proteins	11
The number of binding states	10
The number of binding partners	9

Coloring

Unicolor (beige)

The number of binding partners

Group

Binding state	Binding partners
	3PRX (CHAIN: A)
1	Q91132 D3JIB2
2	Monomeric state
3	Q91132
4	P01031
5	Q6GJP2
6	A6QE84
7	P13671
8	5I5K 5I5K
9	P01031 7AD6
10	P01031 7AD7

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Only interaction residues

#binding
partners

	4
	3
	2
	1
	0

Sequence information

MGLLGILCFL

IFLGKTWGQE

QTYVISAPKI

FRVGASENIV

IQVYGYTEAF

DATISIKSYP

DKKFSYSSGH

VHLSSENKFQ

NSAILTIQPK

QLPGGQNPVS

100

101

YVYLEVVSKH

FSKSKRMPIT

YDNGFLFIHT

DKPVYTPDQS

VKVRVYSLND

150

151

DLKPAKRETV

LTFIDPEGSE

VDMVEEIDHI

GIISFPDFKI

PSNPRYGMWT

200

201

IKAKYKEDFS

TTGTAYFEVK

EYVLPHFSVS

IEPEYNFIGY

KNFKNFEITI

250

251

KARYFYNKVV

TEADVYITFG

IREDLKDDQK

EMMQTAMQNT

MLINGIAQVT

300

301

FDSETAVKEL

SYYSLEDLNN

KYLYIAVTVI

ESTGGFSEEA

EIPGIKYVLS

350

351

PYKLNLVATP

LFLKPGIPYP

IKVQVKDSLD

QLVGGVPVTL

NAQTIDVNQE

400

401

TSDLDPSKSV

TRVDDGVASF

VLNLPSGVTV

LEFNVKTDAP

DLPEENQARE

450

451

GYRAIAYSSL

SQSYLYIDWT

DNHKALLVGE

HLNIIVTPKS

PYIDKITHYN

500

501

YLILSKGKII

HFGTREKFSD

ASYQSINIPV

TQNMVPSSRL

LVYYIVTGEQ

550

551

TAELVSDSVW

LNIEEKCGNQ

LQVHLSPDAD

AYSPGQTVSL

NMATGMDSWV

600

601

ALAAVDSAVY

GVQRGAKKPL

ERVFQFLEKS

DLGCGAGGGL

NNANVFHLAG

650

651

LTFLTNANAD

DSQENDEPCK

EILRPRRTLQ

KKIEEIAAKY

KHSVVKKCCY

700

701

DGACVNNDET

CEQRAARISL

GPRCIKAFTE

CCVVASQLRA

NISHKDMQLG

750

751

RLHMKTLLPV

SKPEIRSYFP

ESWLWEVHLV

PRRKQLQFAL

PDSLTTWEIQ

800

801

GVGISNTGIC

VADTVKAKVF

KDVFLEMNIP

YSVVRGEQIQ

LKGTVYNYRT

850

851

SGMQFCVKMS

AVEGICTSES

PVIDHQGTKS

SKCVRQKVEG

SSSHLVTFTV

900

901

LPLEIGLHNI

NFSLETWFGK

EILVKTLRVV

PEGVKRESYS

GVTLDPRGIY

950

951

GTISRRKEFP

YRIPLDLVPK

TEIKRILSVK

GLLVGEILSA

VLSQEGINIL

1000

1001

THLPKGSAEA

ELMSVVPVFY

VFHYLETGNH

WNIFHSDPLI

EKQKLKKKLK

1050

1051

EGMLSIMSYR

NADYSYSVWK

GGSASTWLTA

FALRVLGQVN

KYVEQNQNSI

1100

1101

CNSLLWLVEN

YQLDNGSFKE

NSQYQPIKLQ

GTLPVEAREN

SLYLTAFTVI

1150

1151

GIRKAFDICP

LVKIDTALIK

ADNFLLENTL

PAQSTFTLAI

SAYALSLGDK

1200

1201

THPQFRSIVS

ALKREALVKG

NPPIYRFWKD

NLQHKDSSVP

NTGTARMVET

1250

1251

TAYALLTSLN

LKDINYVNPV

IKWLSEEQRY

GGGFYSTQDT

INAIEGLTEY

1300

1301

SLLVKQLRLS

MDIDVSYKHK

GALHNYKMTD

KNFLGRPVEV

LLNDDLIVST

1350

1351

GFGSGLATVH

VTTVVHKTST

SEEVCSFYLK

IDTQDIEASH

YRGYGNSDYK

1400

1401

RIVACASYKP

SREESSSGSS

HAVMDISLPT

GISANEEDLK

ALVEGVDQLF

1450

1451

TDYQIKDGHV

ILQLNSIPSS

DFLCVRFRIF

ELFEVGFLSP

ATFTVYEYHR

1500

1501

PDKQCTMFYS

TSNIKIQKVC

EGAACKCVEA

DCGQMQEELD

LTISAETRKQ

1550

1551

TACKPEIAYA

YKVSITSITV

ENVFVKYKAT

LLDIYKTGEA

VAEKDSEITF

1600

1601

IKKVTCTNAE

LVKGRQYLIM

GKEALQIKYN

FSFRYIYPLD

SLTWIEYWPR

1650

1651

DTTCSSCQAF

LANLDEFAED

IFLNGC

1700

Variants

Residue	AA	Source / dbSNP	Clinical Significance	Allele Frequency (> 0.0001)	AlphaMissense pathogenicity	Disease name
49_ALA	VAL	8.3kJPN chr9:123808581 -	-	0.0001	0.1205	-
72_HIS	ASN	8.3kJPN chr9:123808513 rs1239344218	-	0.0007	0.045	-
76_GLU	ASP	8.3kJPN chr9:123808499 -	-	0.0001	0.09	-
108_SER	TER	8.3kJPN chr9:123805380 -	-	0.0001	-	-
110_HIS	ASN	gnomAD chr9:123805375 rs143947726	-	0.000358594	0.0946	-
145_VAL	ILE	VAR_038735 rs17216529	LB/B	-	0.0966	-
145_VAL	ILE	gnomAD chr9:123800218 rs17216529	-	0.0779188	0.0966	-
145_VAL	ILE	8.3kJPN chr9:123800218 rs17216529	-	0.2014	0.0966	-
164_ILE	LYS	8.3kJPN chr9:123800160 -	-	0.0001	0.1433	-
171_VAL	ALA	8.3kJPN chr9:123797153 rs185695138	-	0.0013	0.2664	-
176_GLU	TER	ClinVar chr9:123797139 -	Pathogenic	-	-	not provided [MedGen:C3661900]
238_ILE	THR	8.3kJPN chr9:123792720 rs567288479	-	0.0001	0.2738	-
238_ILE	VAL	8.3kJPN chr9:123792721 -	-	0.0001	0.1116	-
239_GLY	SER	gnomAD chr9:123792718 rs369524514	-	0.000692156	0.0762	-
252_ALA	THR	gnomAD chr9:123792679 rs112959008	-	0.000320111	0.4197	-
265_VAL	ILE	8.3kJPN chr9:123789518 rs148539497	-	0.0001	0.1069	-
274_ASP	GLY	8.3kJPN chr9:123789490 -	-	0.0002	0.104	-
277_ASP	GLY	8.3kJPN chr9:123789481 rs1462885944	-	0.0001	0.076	-
280_LYS	ASN	8.3kJPN chr9:123789471 -	-	0.0012	0.6197	-
281_GLU	ASP	8.3kJPN chr9:123789468 rs201821112	-	0.0001	0.1712	-
294_ASN	LYS	gnomAD chr9:123787850 rs774028867	-	0.00015167	0.2251	-
297_ALA	VAL	8.3kJPN chr9:123787842 rs1269751460	-	0.0001	0.2038	-
298_GLN	GLU	gnomAD chr9:123787840 rs149346283	-	0.000111674	0.0619	-
330_ILE	THR	gnomAD chr9:123787743 rs147430470	-	0.000620093	0.0644	-
339_GLU	GLY	8.3kJPN chr9:123785782 -	-	0.0005	0.2908	-
354_LEU	MET	VAR_048822 rs34552775	LB/B	-	0.184	-
354_LEU	MET	gnomAD chr9:123785738 rs34552775	-	0.00529656	0.184	-
372_LYS	ARG	ClinVar chr9:123785683 rs587776846	Pathogenic	-	0.0989	Complement component 5 deficiency [MONDO:MONDO:0012295,MedGen:C0343047,OMIM:609536]
389_THR	ILE	VAR_023946 -	LB/B	-	0.0736	-
390_LEU	VAL	8.3kJPN chr9:123783921 rs763556527	-	0.0001	0.0844	-
405_ASP	HIS	8.3kJPN chr9:123783876 -	-	0.0002	0.1044	-
417_VAL	ALA	8.3kJPN chr9:123783839 -	-	0.0001	0.1993	-
429_THR	LYS	gnomAD chr9:123783803 rs145461325	-	0.000338061	0.0919	-
429_THR	MET	8.3kJPN chr9:123783803 rs145461325	-	0.0001	0.0962	-
449_ARG	GLY	VAR_038736 rs2230213	LB/B	-	0.082	-
449_ARG	GLY	gnomAD chr9:123782415 rs2230213	-	0.0073537	0.082	-
451_GLY	VAL	8.3kJPN chr9:123782408 rs56188613	-	0.0089	0.0738	-
469_TRP	TER	ClinVar chr9:123782353 rs1408798589	Pathogenic/Likely pathogenic	-	-	not provided\|Eculizumab, poor response to [MedGen:C3661900\|MedGen:C3810402,OMIM:615749]; Complement component 5 deficiency [MONDO:MONDO:0012295,MedGen:C0343047,OMIM:609536]
473_HIS	ARG	gnomAD chr9:123782342 rs78640581	-	0.000230679	0.0738	-
475_ALA	VAL	gnomAD chr9:123782336 rs151271738	-	0.000163072	0.0929	-
493_ILE	MET	8.3kJPN chr9:123782281 -	-	0.0001	0.0768	-
506_LYS	GLN	8.3kJPN chr9:123780121 -	-	0.0001	0.2198	-
512_PHE	LEU	8.3kJPN chr9:123780103 -	-	0.0001	0.9029	-
514_THR	MET	8.3kJPN chr9:123780096 rs199664327	-	0.003	0.1361	-
515_ARG	LYS	8.3kJPN chr9:123780093 rs1423414968	-	0.0001	0.073	-
518_PHE	SER	VAR_001996 -	LB/B	-	0.0939	-
560_TRP	TER	ClinVar chr9:123779957 -	Pathogenic	-	-	not provided [MedGen:C3661900]
571_LEU	VAL	gnomAD chr9:123779926 rs147226792	-	0.000223104	0.1718	-
572_GLN	ARG	8.3kJPN chr9:123779922 -	-	0.0001	0.0836	-
599_TRP	TER	8.3kJPN chr9:123779709 -	-	0.0001	-	-
600_VAL	GLU	8.3kJPN chr9:123779707 -	-	0.0001	0.9191	-
605_VAL	LEU	8.3kJPN chr9:123779693 -	-	0.0001	0.2461	-
606_ASP	ASN	gnomAD chr9:123779690 rs775286780	-	0.000214925	0.8225	-
647_HIS	GLN	gnomAD chr9:123778587 rs202080772	-	0.000178981	0.1049	-
647_HIS	GLN	8.3kJPN chr9:123778587 rs202080772	-	0.0004	0.1049	-
673_LEU	ILE	gnomAD chr9:123777519 rs369245774	-	0.000184003	0.0834	-
701_ASP	ASN	8.3kJPN chr9:123776307 rs187925604	-	0.0001	0.1379	-
714_ARG	TER	8.3kJPN chr9:123776268 -	-	0.0001	-	-
734_VAL	ILE	8.3kJPN chr9:123776208 rs771791383	-	0.0005	0.0698	-
756_THR	ILE	8.3kJPN chr9:123770767 rs1456300999	-	0.0003	0.1827	-
802_VAL	ILE	VAR_014574 rs17611	LB/B	-	0.0676	-
802_VAL	ILE	gnomAD chr9:123769200 rs17611	-	0.471368	0.0676	-
802_VAL	ILE	8.3kJPN chr9:123769200 rs17611	-	0.5195	0.0676	-
809_ILE	VAL	8.3kJPN chr9:123768334 rs750127089	-	0.0001	0.1437	-
885_ARG	CYS	VAR_071067 rs373359894	LB/B	-	0.1594	-
885_ARG	HIS	VAR_071068 rs56040400	LB/B	-	0.0728	-
885_ARG	HIS	gnomAD chr9:123759961 rs56040400	-	0.00032607	0.0728	-
885_ARG	HIS	8.3kJPN chr9:123759961 rs56040400	-	0.019	0.0728	-
907_LEU	PHE	8.3kJPN chr9:123759896 rs772788429	-	0.0009	0.1303	-
928_ARG	GLN	VAR_038737 rs41309892	LB/B	-	0.1388	-
928_ARG	TER	ClinVar chr9:123759833 -	Pathogenic	-	-	not provided [MedGen:C3661900]
928_ARG	GLN	gnomAD chr9:123759832 rs41309892	-	0.0022379	0.1388	-
933_GLY	VAL	VAR_038738 rs41309902	LB/B	-	0.9027	-
965_LEU	TER	ClinVar chr9:123753516 -	Pathogenic	-	-	not provided [MedGen:C3661900]
966_ASP	TYR	VAR_048823 rs2230212	LB/B	-	0.2024	-
966_ASP	TYR	gnomAD chr9:123753514 rs2230212	-	0.00474118	0.2024	-
966_ASP	TYR	8.3kJPN chr9:123753514 rs2230212	-	0.0317	0.2024	-
1006_GLY	ALA	8.3kJPN chr9:123751983 rs754352144	-	0.0005	0.2948	-
1010_ALA	VAL	gnomAD chr9:123751971 rs34362143	-	0.000703928	0.1995	-
1011_GLU	ASP	gnomAD chr9:123751967 rs150096192	-	0.000906719	0.5832	-
1015_VAL	ILE	8.3kJPN chr9:123751957 rs765069093	-	0.0001	0.0599	-
1033_ILE	THR	VAR_038739 rs41311881	LB/B	-	0.1485	-
1033_ILE	THR	gnomAD chr9:123751902 rs41311881	-	0.00120578	0.1485	-
1037_ASP	ASN	VAR_038740 rs41311883	LB/B	-	0.062	-
1043_GLN	LYS	VAR_038741 rs41311887	LB/B	-	0.0756	-
1043_GLN	LYS	gnomAD chr9:123751873 rs41311887	-	0.00493069	0.0756	-
1053_MET	LEU	VAR_014575 rs17609	LB/B	-	0.0726	-
1119_LYS	ARG	gnomAD chr9:123744967 rs142341763	-	0.000211822	0.0737	-
1138_ARG	GLN	gnomAD chr9:123744195 rs779879112	-	0.000210836	0.0701	-
1155_ALA	THR	gnomAD chr9:123744145 rs200624729	-	0.000282457	0.1146	-
1183_GLN	ARG	8.3kJPN chr9:123742471 -	-	0.0001	0.0629	-
1214_ARG	LYS	gnomAD chr9:123742378 rs150470491	-	0.000262448	0.061	-
1257_THR	SER	gnomAD chr9:123739072 rs141704823	-	0.000512996	0.1417	-
1291_ILE	VAL	gnomAD chr9:123737203 rs200674959	-	0.00025848	0.0688	-
1310_SER	ASN	VAR_014576 rs17610	LB/B	-	0.0526	-
1310_SER	ASN	gnomAD chr9:123737145 rs17610	-	0.00727767	0.0526	-
1314_ASP	ASN	gnomAD chr9:123737134 rs771283592	-	0.000119307	0.0543	-
1320_LYS	GLN	8.3kJPN chr9:123737116 -	-	-	0.0973	-
1325_ASN	SER	8.3kJPN chr9:123737100 -	-	0.0001	0.0744	-
1336_ARG	LYS	8.3kJPN chr9:123737067 -	-	0.0002	0.0709	-
1365_VAL	ALA	VAR_048824 rs16910245	LB/B	-	0.1312	-
1402_ILE	VAL	gnomAD chr9:123731355 rs142459293	-	0.000469442	0.094	-
1405_CYS	ARG	8.3kJPN chr9:123731346 rs753067140	-	0.0001	0.9527	-
1437_GLU	ASP	VAR_014577 rs17612	LB/B	-	0.069	-
1437_GLU	ASP	gnomAD chr9:123725926 rs17612	-	0.0536851	0.069	-
1437_GLU	ASP	8.3kJPN chr9:123725926 rs17612	-	0.0003	0.069	-
1454_GLN	ARG	8.3kJPN chr9:123725208 -	-	0.0001	0.091	-
1476_ARG	TER	ClinVar chr9:123725027 rs121909588	Pathogenic	-	-	Complement component 5 deficiency\|not provided\|Eculizumab, poor response to [MONDO:MONDO:0012295,MedGen:C0343047,OMIM:609536\|MedGen:C3661900\|MedGen:C3810402,OMIM:615749]; Complement component 5 deficiency [MONDO:MONDO:0012295,MedGen:C0343047,OMIM:609536]
1478_ARG	TRP	gnomAD chr9:123725021 rs138933092	-	0.000143262	0.234	-
1478_ARG	TRP	8.3kJPN chr9:123725021 rs138933092	-	0.0005	0.234	-
1499_HIS	GLN	8.3kJPN chr9:123724956 -	-	0.0001	0.5062	-
1538_GLU	TER	8.3kJPN chr9:123722592 -	-	0.0001	-	-
1540_ASP	ASN	8.3kJPN chr9:123722586 rs755260495	-	0.0004	0.0939	-
1556_GLU	GLY	gnomAD chr9:123722537 rs201704886	-	0.000211261	0.0861	-
1610_GLU	GLY	8.3kJPN chr9:123716080 -	-	0.0001	0.0947	-
1654_CYS	ARG	8.3kJPN chr9:123715088 rs748755309	-	0.0001	0.8161	-
1662_ALA	VAL	8.3kJPN chr9:123715063 -	-	0.0001	0.1261	-
1670_ASP	HIS	8.3kJPN chr9:123715040 rs761048515	-	0.0006	0.4705	-

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Reference

PiSite: a database of protein interaction sites using multiple binding states in the PDB, Miho Higurashi, Takashi Ishida and Kengo Kinoshita, Nucleic Acids Research 2009 37(Database issue):D360-D364

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Sequence information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Variants

Reference

	5I5K	Eculizumab light chain (variable domain)
	5I5K	Eculizumab heavy chain (variable domain)

	Q91132	Cobra venom factor
	D3JIB2	Superantigen-like protein 7

	P01031	Complement C5
	7AD6	K92 knob domain