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The mutations reported from Factor XI are listed below:

Click on the highlighted residues for further information on mutation.


MIFLYQVVHFILFTSVSGECVTQLLKDTCFEGGDITTVFTPSAKYCQVVCTYHPRCLLFT
FTAESPSEDPTRWFTCVLKDSVTETLPRVNRTAAISGYSFKQCSHQISACNKDIYVDLDM
KGINYNSSVAKSAQECQERCTDDVHCHFFTYATRQFPSLEHRNICLLKHTQTGTPTRITK
LDKVVSGFSLKSCALSNLACIRDIFPNTVFADSNIDSVMAPDAFVCGRICTHHPGCLFFT
FFSQEWPKESQRNLCLLKTSESGLPSTRIKKSKALSGFSLQSCRHSIPVFCHSSFYHDTD
FLGEELDIVAAKSHEACQKLCTNAVRCQFFTYTPAQASCNEGKGKCYLKLSSNGSPTKIL
HGRGGISGYTLRLCKMDNECTTKIKPRIVGGTASVRGEWPWQVTLHTTSPTQRHLCGGSI
IGNQWILTAAHCFYGVESPKILRVYSGILNQSEIKEDTSFFGVQEIIIHDQYKMAESGYD
IALLKLETTVNYTDSQRPICLPSKGDRNVIYTDCWVTGWGYRKLRDKIQNTLQKAKIPLV
TNEECQKRYRGHKITHKMICAGYREGGKDACKGDSGGPLSCKHNEVWHLVGITSWGEGCA
QRERPGVYTNVVEYVDWILEKTQAV

Missense

Position* Wild type Mutant Domain PMID
34AspHisApple 17888672
38ValAlaApple 118024374
41ProGlnApple 116835901
41ProLeuApple 116079124
42SerArgApple 119652879
46CysPheApple 115953011
47GlnHisApple 1 
51ThrIleApple 118005151
51ThrProApple 118005151
53HisTyrApple 119652879
56CysArgApple 119598070, 18387979, 11895778
56CysTrpApple 1 
66ProLeuApple 110391209
70ProLeuApple 1 
75  ThrIle  Apple 118248411
76CysArgApple 116835901
76CysPheApple 116835901
76CysTyrApple 118024374
87ProThrApple 116079124
97GlyAlaApple 118839438
99SerTyrApple 119652879
101LysArgApple 115953011
109AlaThr 18515884
110CysGlyApple 216079124
120MetThrApple 216835901, 17549289
122GlyAspApple 212716376
136CysArgApple 220491955
140CysTyrApple 218515884, 16607084
141ThrMetApple 218515884
145HisArgApple 2 
150ThrMetApple 216835901, 19652879
151TyrCysApple 215953011
151TyrSerApple 217549289, 16835901
152AlaProApple 2 
162ArgCysApple 2 
173GlyGluApple 219652879
190LeuProApple 215182578
199AlaVal 18388506
200CysTyrApple 318446632
202ArgGlyApple 319630775, 18327400
206ProSerApple 316607084
216AspAsnApple 3 
230CysArgApple 3 
230CysSerApple 3 
239PheSerApple 316787881
241PheLeuApple 321457405
243SerPheApple 315728123
244GlnArgApple 39787168
246TrpCysApple 310027710
252ArgIleApple 3 
252ArgLysApple 318446632
252ArgSerApple 3 
255CysTyrApple 318024374, 11895778
261GluAspApple 316079124
263GlyGluApple 315953011
266SerAsnApple 311418471
266SerAspApple 319630775
266SerGlnApple 39787168
268ArgCysApple 3 
268ArgHisApple 310630991, 18446632
270LysIleApple 318839438, 15180874
289ValLeu 15842381
301PheLeuApple 41547342, 15026311, 19630775
302LeuAlaApple 418441012
305GluAlaApple 418441012
308IleAlaApple 418441012
308IlePheApple 410391209
308IleThrApple 415953011
315GluLysApple 418024374
320LeuProApple 47888672
322ThrIleApple 47888672
325ValPheApple 419652879
326ArgCysApple 410606881, 19652879
331ThrIleApple 418005151
339CysPheApple 411895778
339CysSerApple 418441012
341GluLysApple 418024374, 19652879, 7888672
347TyrAlaApple 418441012
349LysAlaApple 418441012
354GlyArgApple 414717969
359IleMetApple 419652879
361HisAlaApple 418441012
362GlyArgApple 4 
368GlyAlaApple 419652879, 14717969
368GlyArgApple 4 
368GlyGluApple 415026311, 18839438
369TyrSerApple 415026311, 15842381
373LeuSerApple 419652879
374CysArgApple 419652879
389ValIlePeptidase S119630775, 17971173
390GlyAlaPeptidase S118758779
393AlaValPeptidase S119652879
396ArgCysPeptidase S120829681, 20510102, 19923982, 20860169,
399TrpLeuPeptidase S1 
400ProLeuPeptidase S1 
403ValMetPeptidase S121457405
404ThrAsnPeptidase S19401068
406HisProPeptidase S118388506
407ThrProPeptidase S1 
408ThrProPeptidase S1 
416CysTyrPeptidase S115728123, 19652879, 18024374
418GlySerPeptidase S1 
418GlyValPeptidase S117370644, 15026311
419SerAlaPeptidase S1 
425TrpCysPeptidase S118388506, 15026311
428ThrIlePeptidase S119652879
430AlaSerPeptidase S119652879
430AlaThrPeptidase S118388506, 18839438
430AlaValPeptidase S115634276
443ArgCysPeptidase S1 
445TyrCysPeptidase S118024374
446SerGlyPeptidase S118446632
451GlnGluPeptidase S117581330
460PheValPeptidase S17669672
478GlyArgPeptidase S112716376, 15842381
493ThrIlePeptidase S115968392
500CysArgPeptidase S119652879
500CysTrpPeptidase S119652879
503SerProPeptidase S118005151
511TyrHisPeptidase S111895778
515TrpCysPeptidase S116079124
515TrpGlyPeptidase S120491955
516ValMetPeptidase S118832909
519TrpCysPeptidase S115060426
536LysAsnPeptidase S111127865
538ProLeuPeptidase S117229051, 19652879, 19630775
545CysTyrPeptidase S118024374
555GluGlyPeptidase S119630775
562GlySerPeptidase S119652879
565GluLysPeptidase S115953011, 18758779
573GlyGluPeptidase S115090552, 15456490
574AspGlyPeptidase S1 
581CysPhePeptidase S1 
587TrpSerPeptidase S115026311
593ThrMetPeptidase S117549289, 19630775
594SerArgPeptidase S115842381, 15634276
596GlyCysPeptidase S118515884
597GluLysPeptidase S116607084
608TyrHisPeptidase S118005151
617TrpArgPeptidase S115996948
618IleSerPeptidase S115953011

Insertion

Mutation Codon Exon/Intron Domain PMID
 192_193insG    Exon 12  Apple 1  14717969
 ins[AT(A)(28)TAAAG(A)(26)GGAAATAATAATTCA]    Exon 3    15566468
 137insG        14717969
 307insAAGCAAT    Exon 13    11127865
 501/502 Gins        17581330

Deletion

Mutation Codon Exon/Intron Domain PMID
 delG  285      11895778
 962delG    Exon 9  Apple 4  15953011
 73-86del      Apple 1  18024374
 78-80delGGA      Apple 1  19718484
 962delG  288  Exon 9  Apple 4  15953011
 960-961delGT    Exon 9    16607084
 687_692delTCGACA    Exon 7    16519703
 IVS J-4delGTTG        15946525
 643-648delATCGAC    Exon 7    15531455
 1072delA        11127865
 14-bpdelAAGgtaacagagtg  554  Exon 14 / Iintron N    8807341

Nonsense

Mutation* Codon Exon/Intron Domain PMID
 Gln263X        21668437
 Gln244X        21668437
 Cys56X      Apple 1  16905957
 Arg72X      Apple 1  16905957
 Gln106X      Linker Region  14717969
 Gln134X      Apple 2  18515884
 Glu135X      Apple 2  20301226, 19937244, 20331761, 20664902, 21429375, 20231421,
 Cys136X      Apple 2  20301226, 19937244, 20331761, 20664902, 21429375, 20231421,
 Cys146X    Exon 5  Apple 2  7669672, 15953011, 9326232
 Arg228X      Apple 3  14717969
 Trp246X      Apple 3  15182578
 Gln281X    Exon 8  Apple 3  19347998
 Cys327X      Apple 4  18515884
 Tyr369X    Exon 10  Apple 4  14508802
 Trp401X      Peptidase S1  12697122
 Glu465X    Exon 12  Peptidase S1  12879434
 Trp519X      Peptidase S1  11122101
 Cys599X      Peptidase S1  14717969
 Tyr608X    Exon 15  Peptidase S1  15953011

Polymorphism

Mutation* Codon Exon/Intron Domain PMID
 Tyr35His      Apple 1  19652879
 Pro66Leu      Apple 1  10391209
 Asp143Asp      Apple 2  11127865
 Ile308Phe      Apple 4  15953011
 Arg396Cys      Peptidase S1  17549289

Splice site

Mutation Codon Exon/Intron Domain PMID
 IVS6+3A>G        11895778, 18327400
 IVS2, 55+6T>G, +10insAG      Apple 1  18839438
 IVS3,218+2T>A      Introgenic Region  18839438
 325+1G>A      Introgenic Region  16835901, 18446632
 325G>A    Exon 4  Apple 2  18327400
 1026G>T        11127865
 218+126A>G      Introgenic Region  14717969
 301_307dupAAGCAAT      Apple 1  11127865

* The positions are based on the protein sequence inclusive of the signal peptide if present.


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©Biomedical Informatics Centre, NIRRH, Mumbai