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The mutations reported for Factor I are listed below:

Factor I has three chains - Alpha, Beta and Gamma

Alpha chain

Click on the highlighted residues for further information on mutation.


MFSMRIVCLVLSVVGTAWTADSGEGDFLAEGGGVRGPRVVERHQSACKDSDWPFCSDEDW
NYKCPSGCRMKGLIDEVNQDFTNRINKLKNSLFEYQKNNKDSHSLTTNIMEILRGDFSSA
NNRDNTYNRVSEDLRSRIEVLKRKVIEKVQHIQLLQKNVRAQLVDMKRLEVDIDIKIRSC
RGSCSRALAREVDLKDYEDQQKQLEQVIAKDLLPSRDRQHLPLIKMKPVPDLVPGNFKSQ
LQKVPPEWKALTDMPQMRMELERPGGNEITRGGSTSYGTGSETESPRNPSSAGSWNSGSS
GPGSTGNRNPGSSGTGGTATWKPGSSGPGSTGSWNSGSSGTGSTGNQNPGSPRPGSTGTW
NPGSSERGSAGHWTSESSVSGSTGQWHSESGSFRPDSPGSGNARPNNPDWGTFEEVSGNV
SPGTRREYHTEKLVTSKGDKELRTGKEKVTSGSTTTTRRSCSKTVTKTVIGPDGHKEVTK
EVVTSEDGSDCPEAMDLGTLSGIGTLDGFRHRHPDEAAFFDTASTGKTFPGFFSPMLGEF
VSETESRGSESGIFTNTKESSSHHPGIAEFPSRGKSSSYSKQFTSSTSYNRGDSTFESKS
YKMADEAGSEADHEGTHSTKRGHAKSRPVRDCDDVLQTHPSGTQSGIFNIKLPGSSKIFS
VYCDQETSLGGWLLIQQRMDGSLNFNRTWQDYKRGFGSLNDEGEGEFWLGNDYLHLLTQR
GSVLRVELEDWAGNEAYAEYHFRVGSEAEGYALQVSSYEGTAGDALIEGSVEEGAEYTSH
NNMQFSTFDRDADQWEENCAEVYGGGWWYNNCQAANLNGIYYPGGSYDPRNNSPYEIENG
VVWVSFRGADYSLRAVRMKIRPLVTQ

Missense

Position* Wild type Mutant Domain PMID
28LeuPro 12706644
30GluGly 7903170
31GlyVal 11914657
32GlyGlu 21112076
35ArgCys 20829681, 20510102, 19923982
35ArgHis 20829681, 20510102, 19923982
35ArgSer 19923982
36GlyVal 8259537
38ArgGly 11204560, 10605955
39ValAsp 8675656
64CysPhe 18676163
70MetArg 17531448
125AsnAsp 19718467
160ArgSer 1634621
262GluAsp 19417632
331ThrAla 20010469
347GlnPro 16735602
453SerAsn 1675636
458ArgCys 18485090
496AspAsn 17849064
513HisPro 19572065
515AspAsn 17849064
545GluVal 19109585, 18500534, 10825402, 15523923
551SerCys 10959699
557ThrLys 19073821
559GluVal 19073821
571ProHis 19073821
573 Arg Leu 10036586, 9818055, 8097946
573ArgCys 8473507
573ArgLeu 10036586, 9818055, 8097946

Insertion

Mutation Codon Exon/Intron Domain PMID
 34insC    Exon 1    10891444
 117-bp insertion        12871327

Deletion

Mutation Codon Exon/Intron Domain PMID
 1215delT    Exon 3, Exon 4    17531448
 delTCTT    Exon 1    10602365
 C1537delA  494  Exon 5    19572064
 4110delA        17179831
 IVS3+1_+4delGTAA    Intron 3    12406899
 1215delT    Exon 3, Exon 4    11739173, 17531448
 3121delAA    Exon 4    10891444
 4329delC    Exon 5    10891444
 4904delG  524      8639778
 4758-4770del        8611457
 65682-69828del    Exon 1    17393016
 1787-1789del3ins12    Exon 1    17393016
 3120-3122delAA  140  Exon 4    12695755
 1846 del A    exon 5    21245743

Nonsense

Mutation* Codon Exon/Intron Domain PMID
 Gly32X    Exon 2    11739173
 Ser119X    Exon 3    11739173
 Arg129X    Exon 4    11739173
 Gln150X    Exon 4    15946522
 Arg168X    Exon 4    11739173, 10887149
 Arg178X        19468208
 Gln240X        20301226, 19937244, 20331761, 20664902, 21429375, 20231421,
 Trp248X        17393016
 Gly316X    Exon 5    10891444
 Trp334X    Exon 5    18388508
 Trp334X    Exon 5    10891444
 Gln347X        14615374
 Lys480X        1391954
 Ser485X        16406498
 Glu486X        15166913

Polymorphism

Mutation* Codon Exon/Intron Domain PMID
 Thr331Ala        17433418, 10318664

Splice site

Mutation Codon Exon/Intron Domain PMID
 3200+1G>T        17179831
 IVS4+1G>T    Intron 4    14615374, 12406899, 11460507
 IVS1+3A>G    Intron 1    12406899
 IVS3+1_+4delGTAA    Intron 3    12406899

Beta chain

Click on the highlighted residues for further information on mutation.


MKRMVSWSFHKLKTMKHLLLLLLCVFLVKSQGVNDNEEGFFSARGHRPLDKKREEAPSLR
PAPPPISGGGYRARPAKAAATQKKVERKAPDAGGCLHADPDLGVLCPTGCQLQEALLQQE
RPIRNSVDELNNNVEAVSQTSSSSFQYMYLLKDLWQKRQKQVKDNENVVNEYSSELEKHQ
LYIDETVNSNIPTNLRVLRSILENLRSKIQKLESDVSAQMEYCRTPCTVSCNIPVVSGKE
CEEIIRKGGETSEMYLIQPDSSVKPYRVYCDMNTENGGWTVIQNRQDGSVDFGRKWDPYK
QGFGNVATNTDGKNYCGLPGEYWLGNDKISQLTRMGPTELLIEMEDWKGDKVKAHYGGFT
VQNEANKYQISVNKYRGTAGNALMDGASQLMGENRTMTIHNGMFFSTYDRDNDGWLTSDP
RKQCSKEDGGGWWYNRCHAANPNGRYYWGGQYTWDMAKHGTDDGVVWMNWKGSWYSMRKM
SMKIRPFFPQQ

Missense

Position* Wild type Mutant Domain PMID
44ArgCys 1565641, 10695765
45GlyCys 2018836, 7493975
71TyrAsn 21301788
74ArgCys 1565641
98AlaThr 1634610, 11434947
178LysAsn 12573244
190AsnSer 10572095
196ArgCys 11468164
265ProLeuFibrinogen C-terminal10688828
267ArgSerFibrinogen C-terminal20640913
285ArgHisFibrinogen C-terminal12573244
294ArgGlyFibrinogen C-terminal16953282
346AspTyrFibrinogen C-terminal11307813
356TyrCysFibrinogen C-terminal17849064
377GlyArgFibrinogen C-terminal16403286
381AsnLysFibrinogen C-terminal20640913
383LeuArgFibrinogen C-terminal10666208
430GlyAspFibrinogen C-terminal10666208
444GlySerFibrinogen C-terminal12893758, 15197468
449GlyValFibrinogen C-terminal16732370
464GlyAspFibrinogen C-terminal16670068
467TrpGlyFibrinogen C-terminal14559115

Deletion

Mutation Codon Exon/Intron Domain PMID
 2397-247del    Exon 2    3156856
 7972delG        15946523

Nonsense

Mutation* Codon Exon/Intron Domain PMID
 Arg17X    Exon 2    16403286, 12161363
 Trp47X    Exon 2    12893758
 Tyr71X        14629469
 Tyr266X      Fibrinogen C-terminal  12871327
 Trp296X      Fibrinogen C-terminal  17393016
 Trp467X      Fibrinogen C-terminal  12511408
 Trp470X      Fibrinogen C-terminal  12353071

Polymorphism

Mutation* Codon Exon/Intron Domain PMID
 G/A-455        10378457, 9409270
 C/T-148  413      10378457
 G/A+448        10378457
 G/A-854        19954614
 C/T-249        19954614
 G455A        20051843, 19906129, 19395327

Splice site

Mutation Codon Exon/Intron Domain PMID
 IVS6+13C>T    Intron 6    12393540
 IVS7+1G>T    Intron 7    12393540
 IVS6Delta4b        20580695

Gamma chain

Click on the highlighted residues for further information on mutation.


MSWSLHPRNLILYFYALLFLSSTCVAYVATRDNCCILDERFGSYCPTTCGIADFLSTYQT
KVDKDLQSLEDILHQVENKTSEVKQLIKAIQLTYNPDESSKPNMIDAATLKSRKMLEEIM
KYEASILTHDSSIRYLQEIYNSNNQKIVNLKEKVAQLEAQCQEPCKDTVQIHDITGKDCQ
DIANKGAKQSGLYFIKPLKANQQFLVYCEIDGSGNGWTVFQKRLDGSVDFKKNWIQYKEG
FGHLSPTGTTEFWLGNEKIHLISTQSAIPYALRVELEDWNGRTSTADYAMFKVGPEADKY
RLTYAYFAGGDAGDAFDGFDFGDDPSDKFFTSHNGMQFSTWDNDNDKFEGNCAEQDGSGW
WMNKCHAGHLNGVYYQGGTYSKASTPNGYDNGIIWATWKTRWYSMKKTTMKIIPFNRLTI
GEGQQHHLGGAKQVRPEHPAETEYDSLYPEDDL

Missense

Position* Wild type Mutant Domain PMID
108AlaGly 19300242, 15795540, 11019970
179CysArgFibrinogen C-terminal10590057, 15197468
191GlyArgFibrinogen C-terminal11435303
226GlyValFibrinogen C-terminal17650452
241TrpAlaFibrinogen C-terminal11460501
253TrpCysFibrinogen C-terminal16141000
253TrpGlyFibrinogen C-terminal17080227
256AsnAspFibrinogen C-terminal20580674
265GlnHisFibrinogen C-terminal15748264
288TyrCysFibrinogen C-terminal18485115
289AlaValFibrinogen C-terminal15583736
294GlyGluFibrinogen C-terminal8639838
301ArgSerFibrinogen C-terminal10404772
305AlaAspFibrinogen C-terminal10691848
306TyrCysFibrinogen C-terminal10702704, 9576177
309GlyAspFibrinogen C-terminal11986213
310GlyArgFibrinogen C-terminal10880389, 15197468
315AlaValFibrinogen C-terminal20829681, 20510102, 19923982, 20860169,
318GlyValFibrinogen C-terminal2257302, 10064005
333HisTyrFibrinogen C-terminal20829681, 20510102, 19923982, 20860169,
334AsnIleFibrinogen C-terminal2328317
334AsnLysFibrinogen C-terminal2328317
335GlyAspFibrinogen C-terminal11986213
336MetIleFibrinogen C-terminal12669117
336MetThrFibrinogen C-terminal20829681, 20510102, 19923982, 20860169,
339SerArgFibrinogen C-terminal20126833
339SerAsnFibrinogen C-terminal20829681, 20510102, 19923982, 20860169
340ThrProFibrinogen C-terminal20666993
344AspTyrFibrinogen C-terminal10613648
344AspValFibrinogen C-terminal18832913
345AsnAspFibrinogen C-terminal12669117
345AsnSerFibrinogen C-terminal18676163, 16363237
351AsnIleFibrinogen C-terminal19404553
352CysSerFibrinogen C-terminal14996011
352CysTyrFibrinogen C-terminal15735819, 16363258, 12669117
353AlaThrFibrinogen C-terminal15070682
355GlnArgFibrinogen C-terminal2738036
356AspTyrFibrinogen C-terminal2819242
356AspValFibrinogen C-terminal3708159
358SerCysFibrinogen C-terminal8080993
361TrpArgFibrinogen C-terminal20838743
362MetIleFibrinogen C-terminal20860169
363AsnLysFibrinogen C-terminal8400260
367AlaAspFibrinogen C-terminal20829681, 20510102, 19923982, 20860169,
367AlaThrFibrinogen C-terminal17849064
367AlaValFibrinogen C-terminal18000621
371AsnAspFibrinogen C-terminal20860169
371AsnSerFibrinogen C-terminal18676163
375AlaThrFibrinogen C-terminal19923982
377GlySerFibrinogen C-terminal17938819, 17331136
378GlyCysFibrinogen C-terminal21057694
379ThrProFibrinogen C-terminal21057694
380TyrCysFibrinogen C-terminal12669117
384SerCysFibrinogen C-terminal8080993
387AsnLysFibrinogen C-terminal12695754
389TyrAsnFibrinogen C-terminal19718467
390AspHisFibrinogen C-terminal8822581
397ThrIleFibrinogen C-terminal20829681, 20510102, 19923982, 20860169,
399AsnThrFibrinogen C-terminal20589319
401ArgGlyFibrinogen C-terminal1733971
401ArgTrpFibrinogen C-terminal16819336, 16615976, 12198657, 15197468
404SerProFibrinogen C-terminal15632207
406LysAsnFibrinogen C-terminal9401066
437$ValIle 18521501

Deletion

Mutation Codon Exon/Intron Domain PMID
 319-320del        11460499
 6bp deletion  319-320  Exon 8    2071611
 7690-7704del    Exon 8    17883696
 4209delA    Exon 5    12161363
 4220delT    Exon 5    12161363
 318-319del        19923982

Nonsense

Mutation* Codon Exon/Intron Domain PMID
 Arg134X        15284111
 Glu257X      Fibrinogen C-terminal  10928469
 Lys111X    exon4    20135062

Polymorphism

Mutation* Codon Exon/Intron Domain PMID
 Ile144Lys        15946523

Splice site

Mutation Codon Exon/Intron Domain PMID
 IVS6-320A>T        19551918
 IVS-3+G>A        11001903
 1876+5G>A    Intron 1    11001902
 IVS3-2G        20580695

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

$ The amino acid position corresponds to Isoform of factor 1 gamma chain.


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