PDB ID 2PMW     CHAIN B
Protein name Proprotein convertase subtilisin/kexin type 9
Uniprot Accession Q5SZQ2
The number of similar proteins 16
The number of binding states 5
The number of binding partners 5

Coloring

Unicolor (beige)

The number of binding partners

Group

Binding
state		 Binding partners
2PMW (CHAIN: B)
1 Q8NBP7  
2 Q8NBP7   Q59FQ1  
3 Q8NBP7   P01130  
4 Q8NBP7   3SQO   3SQO  
5 Q8NBP7   5VL7   5VL7  

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Only interaction residues
#binding
partners
  5
  4
  3
  2
  1
  0

Sequence information

1   SIPWNLERIT   PPRYRADEYQ   PPDGGSLVEV   YLLDTSIQSD   HREIEGRVMV   50
51   TDFENVPEED   GTRFHRQASK   CDSHGTHLAG   VVSGRDAGVA   KGASMRSLRV   100
101   LNCQGKGTVS   GTLIGLEFIR   KSQLVQPVGP   LVVLLPLAGG   YSRVLNAACQ   150
151   RLARAGVVLV   TAAGNFRDDA   CLYSPASAPE   VITVGATNAQ   DQPVTLGTLG   200
201   TNFGRCVDLF   APGEDIIGAS   SDCSTCFVSQ   SGTSQAAAHV   AGIAAMMLSA   250
251   EPELTLAELR   QRLIHFSAKD   VINEAWFPED   QRVLTPNLVA   ALPPSTHGAG   300
301   WQLFCRTVWS   AHSGPTRMAT   AVARCAPDEE   LLSCSSFSRS   GKRRGERMEA   350
351   QGGKLVCRAH   NAFGGEGVYA   IARCCLLPQA   NCSVHTAPPA   EASMGTRVHC   400
401   HQQGHVLTGC   SSHWEVEDLG   THKPPVLRPR   GQPNQCVGHR   EASIHASCCH   450
451   APGLECKVKE   HGIPAPQEQV   TVACEEGWTL   TGCSALPGTS   HVLGAYAVDN   500
501   TCVVRSRDVS   TTGSTSEGAV   TAVAICCRSR   HLAQASQELQ     550

Variants

Residue AA Source dbSNP Clinical
Significance
 Allele
Frequency
(> 0.0001)		 Disease name
153_SER ASN 8.3kJPN
chr1:55512254 		-
- 0.0001 -
209_PRO LEU 8.3kJPN
chr1:55518053 		-
- 0.0001 -
219_GLN GLU 8.3kJPN
chr1:55518082 		rs778617372
- 0.0001 -
233_VAL ALA 8.3kJPN
chr1:55518363 		-
- 0.0001 -
236_GLY SER 8.3kJPN
chr1:55518371 		rs149489325
- 0.0001 -
237_ARG TRP gnomAD
chr1:55518374 		rs148195424
- 0.000718598 -
237_ARG TRP 8.3kJPN
chr1:55518374 		rs148195424
- 0.0001 -
241_VAL MET 8.3kJPN
chr1:55518386 		rs147478188
- 0.0001 -
246_SER ASN 8.3kJPN
chr1:55518402 		-
- 0.0001 -
248_ARG CYS 8.3kJPN
chr1:55518407 		rs779824389
- 0.0002 -
253_LEU PHE gnomAD
chr1:55518422 		rs72646508
- 0.000192791 -
263_GLY SER 8.3kJPN
chr1:55518452 		rs200146448
- 0.007 -
264_THR ILE 8.3kJPN
chr1:55518456 		rs201789841
- 0.0051 -
279_PRO THR gnomAD
chr1:55521701 		rs72646509
- 0.000308363 -
298_ASN ASP gnomAD
chr1:55521758 		rs575003139
- 0.000108556 -
310_VAL MET 8.3kJPN
chr1:55521794 		rs1200999906
- 0.0001 -
323_CYS SER 8.3kJPN
chr1:55521834 		rs1307302473
- 0.0001 -
354_ASN ILE ClinVar
chr1:55523068 		rs1553137543
Likely pathogenic - Hypercholesterolemia, familial, 1 [MONDO:MONDO:0007750,MedGen:C0745103,OMIM:143890,Orphanet:391665]
357_ARG CYS gnomAD
chr1:55523076 		rs148562777
- 0.00015117 -
357_ARG CYS 8.3kJPN
chr1:55523076 		rs148562777
- 0.0001 -
367_ASP HIS gnomAD
chr1:55523106 		rs141867978
- 0.000230776 -
374_ASP TYR ClinVar
chr1:55523127 		rs137852912
Pathogenic/Likely pathogenic - Hypercholesterolemia, autosomal dominant, 3|Hypercholesterolemia, familial, 1 [MONDO:MONDO:0011369,MedGen:C1863551,OMIM:603776|MONDO:MONDO:0007750,MedGen:C0745103,OMIM:143890,Orphanet:391665]
374_ASP HIS ClinVar
chr1:55523127 		rs137852912
Pathogenic - Hypercholesterolemia, familial, 1|Hypercholesterolemia, autosomal dominant, 3 [MONDO:MONDO:0007750,MedGen:C0745103,OMIM:143890,Orphanet:391665|MONDO:MONDO:0011369,MedGen:C1863551,OMIM:603776]
378_CYS TRP 8.3kJPN
chr1:55523141 		rs776752113
- 0.0001 -
391_HIS ASN gnomAD
chr1:55523178 		rs146471967
- 0.000162615 -
392_VAL MET ClinVar
chr1:55523181 		rs1254346075
Pathogenic - Hypercholesterolemia, familial, 1 [MONDO:MONDO:0007750,MedGen:C0745103,OMIM:143890,Orphanet:391665]
412_ARG LYS 8.3kJPN
chr1:55523763 		rs775522541
- 0.0001 -
417_HIS GLN gnomAD
chr1:55523779 		rs143275858
- 0.000214927 -
424_ILE VAL 8.3kJPN
chr1:55523798 		rs759250273
- 0.0092 -
425_ASN SER gnomAD
chr1:55523802 		rs28362261
- 0.00122609 -
428_TRP TER 8.3kJPN
chr1:55523812 		-
- 0.0007 -
443_ALA THR gnomAD
chr1:55523855 		rs28362263
- 0.00719433 -
465_SER TRP ClinVar
chr1:55524211 		-
Likely pathogenic - Hypercholesterolemia, autosomal dominant, 3 [MONDO:MONDO:0011369,MedGen:C1863551,OMIM:603776]
467_PRO LEU 8.3kJPN
chr1:55524217 		-
- 0.0001 -
468_THR ALA ClinVar
chr1:55524219 		rs1553137693
Pathogenic - Hypercholesterolemia, familial, 1 [MONDO:MONDO:0007750,MedGen:C0745103,OMIM:143890,Orphanet:391665]
469_ARG TRP gnomAD
chr1:55524222 		rs141502002
- 0.00060473 -
471_ALA SER ClinVar
chr1:55524228 		rs1553137699
Pathogenic - Hypercholesterolemia, familial, 1 [MONDO:MONDO:0007750,MedGen:C0745103,OMIM:143890,Orphanet:391665]
474_VAL ILE gnomAD
chr1:55524237 		rs562556
- 0.856674 -
474_VAL ILE 8.3kJPN
chr1:55524237 		rs562556
- 0.9687 -
495_ARG TRP 8.3kJPN
chr1:55524300 		rs758999339
- 0.0001 -
496_ARG GLN gnomAD
chr1:55524304 		rs139669564
- 0.000254051 -
499_ARG CYS gnomAD
chr1:55524312 		rs201395805
- 0.000126901 -
499_ARG CYS 8.3kJPN
chr1:55524312 		rs201395805
- 0.0002 -
504_GLY TRP 8.3kJPN
chr1:55525165 		rs374455190
- 0.0001 -
510_ARG TRP 8.3kJPN
chr1:55525183 		rs751649334
- 0.0001 -
514_ALA THR 8.3kJPN
chr1:55525195 		rs1447116791
- 0.0001 -
522_ALA THR 8.3kJPN
chr1:55525219 		rs777300852
- 0.0001 -
536_VAL ILE gnomAD
chr1:55525261 		rs776367625
- 0.000138509 -
549_ARG PRO 8.3kJPN
chr1:55525301 		-
- 0.0001 -
553_HIS ARG gnomAD
chr1:55525313 		rs28362270
- 0.000732503 -
554_GLN GLU gnomAD
chr1:55525315 		rs149311926
- 0.00017853 -
589_VAL MET 8.3kJPN
chr1:55527131 		rs372586224
- 0.0002 -
591_HIS GLN gnomAD
chr1:55527139 		rs529912877
- 0.000140526 -
592_ARG THR 8.3kJPN
chr1:55527141 		-
- 0.0001 -
598_ALA THR gnomAD
chr1:55527158 		rs367606156
- 0.000103727 -
598_ALA THR 8.3kJPN
chr1:55527158 		rs367606156
- 0.0002 -
616_PRO LEU 8.3kJPN
chr1:55527213 		rs755750316
- 0.0001 -
619_GLN PRO gnomAD
chr1:55527222 		rs28362277
- 0.00096086 -
629_GLY ASP 8.3kJPN
chr1:55529064 		rs1261496250
- 0.0007 -
636_SER ARG ClinVar
chr1:55529084 		rs2100343401
Likely pathogenic - Hypercholesterolemia, autosomal dominant, 3 [MONDO:MONDO:0011369,MedGen:C1863551,OMIM:603776]
644_VAL ILE 8.3kJPN
chr1:55529108 		rs143291739
- 0.0035 -
659_ARG GLY 8.3kJPN
chr1:55529153 		rs147182054
- 0.0001 -
670_GLY GLU gnomAD
chr1:55529187 		rs505151
- 0.947698 -
670_GLY GLU 8.3kJPN
chr1:55529187 		rs505151
- 0.9528 -
675_VAL ILE 8.3kJPN
chr1:55529201 		rs760981278
- 0.0001 -
680_ARG TRP gnomAD
chr1:55529216 		rs533555352
- 0.000310126 -
680_ARG LEU 8.3kJPN
chr1:55529217 		-
- 0.0001 -
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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

COPYRIGHTc2008-2019 Miho Higurashi, Takashi Ishida and Kengo Kinoshita. All rights reserved.