| Listing of all reported Polymorphisms in the FVIII Gene | ||||||||||||
| 08-May-12 | ||||||||||||
| Polymorphic nucleotide substitutions found in the factor VIII gene of normal subjects or patients with haemophilia A. | ||||||||||||
| NOTE: THERE WAS A VERY IMPORTANT PAPER ON FVIII POLYMORPHISMS PUBLISHED IN MAY 2007 | ||||||||||||
| Viel et al, (2007) Blood 109, 3713-3724. Please consult this for important updates on FVIII polymorphisms. | ||||||||||||
| Material from this paper has not been added to the listings below, which is maintained in its pre-2007 state. | ||||||||||||
| The initial version of this listing was compiled by Dr Anne Goodeve at the Division of Genomic Medicine, University of Sheffield. | ||||||||||||
| Notes: | ||||||||||||
| Southern: Southern blotting; PCR: polymerase chain reaction; ASO: allele-specific oligonucleotide hybridisation | ||||||||||||
| Nucleotide number and codon number after Gitschier et al (1994) | ||||||||||||
| Alleles: for diallelic polymorphisms, the most common allele is listed first. Alleles are referred to by either size of observed DNA fragments in kb, whether a restriction enzyme cuts (+) or does not (-), or the allelic DNA sequence. | ||||||||||||
| Frequency: ?, no frequency data available. All frequencies are for Caucasian populations, unless shown by * (these latter are for Oriental (Chinese or Japanese) populations. | ||||||||||||
| 1. Intronic Polymorphisms | ||||||||||||
| Detection By: | Exon/Intron | Location | Sequence | Alleles | Frequency | References | Date added to HADB | |||||
| Southern/TaqI | 5' flanking | 17kb 5' | - | 9.5/4.0 | 0.72/0.28 | Kenwrick et al (1991) | Dec-91 | |||||
| MPCR-CSGE | IVS3 | (+7) insT | - | - | absent in at least 50 HA X chrs | Jayandharan et al (2005) | Aug-07 | |||||
| MPCR-CSGE | IVS5 | (-23) delC | - | - | absent in at least 50 HA X chrs | Jayandharan et al (2005) | Aug-07 | |||||
| MPCR-CSGE | IVS5 | (-42) delC | - | - | absent in at least 50 HA X chrs | Jayandharan et al (2005) | Aug-07 | |||||
| PCR/ASO | IVS 7, nt27 | - | - | A/G | 0.52/0.48 | Kogan & Gitschier(1990) | Dec-90 | |||||
| direct sequencing | IVS 7, nt32 | - | - | G>C | ? | Klopp et al (2002) | Dec-02 | |||||
| direct sequencing | IVS 7, nt32 | - | - | G>C | ? | Klopp et al (2002) | Dec-02 | |||||
| MPCR-CSGE | IVS9 | (+18) T>G | - | - | absent in at least 50 HA X chrs | Jayandharan et al (2005) | Aug-07 | |||||
| PCR/(CA)n repeat | IVS 13 | - | - | >10 | 25(0); 24(0.013); 23(0.05); 22(0.11); 21(0.29); 20(0.45); 19(0.07); 18(0.006); 17(0); 16(0.006) | Lalloz et al (1991) | Dec-91 | |||||
| ? | IVS 14 cDNA 5219+3 | - | - | A/G | ? | Higuchi et al (1990) | Dec-90 | |||||
| PCR/BclI | IVS 18 | - | - | T+/A- | 0.71/0.29 | Kogan et al (1987) | Dec-87 | |||||
| PCR/HindIII | IVS 19, nt 103 | - | - | -/+ | 0.75/0.25 | Graham et al (1990) | Dec-90 | |||||
| PCR/(GT)n(AG)n repeat | IVS 22 | - | - | >6 | 28(0.013); 27(0); 26(0.667); 25(0.307); 24(0); 23(0.013); | Lalloz et al (1994) | Dec-94 | |||||
| Southern/XbaI | IVS 22 | - | - | 4.8/6.2 | 0.59/0.41 | Wion et al (1986) | Dec-86 | |||||
| Southern/MspI | IVS 22 | - | - | 3.8/4.0 | 0.65/0.35* | Inaba et al (1990) | Dec-90 | |||||
| Southern/BglI | IVS 25 | - | - | 5.0/20.0 | 0.90/0.10 | Antonarakis et al (1985) | Dec-85 | |||||
| Southern/MspI | 3' flanking | - | - | 7.5/4.3+3.2 | 0.68/0.32 | Youssoufian et al (1987) | Dec-87 | |||||
| 2. Exonic Polymorphisms | ||||||||||||
| Please note that aminoacid numbering given below is for the mature processed protein, as used almost universally in FVIII studies. | ||||||||||||
| To convert to HGVS-type aminoacid numbering (where initiator Met =+1) add 19 to aminoacid numbers. | ||||||||||||
| Exon/Intron | Nucleotide No. | Codon | Nucleotide Change | Aminoacid Change | FVIII:C (%) | FVIII:Ag (%) | Alleles | Frequency | Comments | References | Date added to HADB | |
| 1 | 102 | 15 | GAC GAT | Asp Asp | ? | ? | C/T | ? | - | Higuchi et al (1990) | Dec-90 | |
| 2 | 224 | 56 | GAT GTT | Asp Val | ? | ? | A/T | ? | - | Toole et al (1984); Lin et al (1993) | Dec-93 | |
| 7 | ? | 285 | TCC TCT | Ser Ser | <1 | ? | C/T | ? | also has missense V234F | Freson et al (1998) | Apr-99 | |
| 8 | ? | 343 | GCG GCA | Ala Ala | <1 | <1 | G/A | ? | also has Intron 22 Inv | Pieneman et al (1995) | Jun-96 | |
| 8 | ? | 343 | GCG GCA | Ala Ala | 27 | ? | G/A | ? | carrier female also has A296T | d'Oiron et al (2000) | Aug-01 | |
| 10 | 1508 | 484 | CGT CAT | Arg His | 1 | ? | G/A | ? | also has causative Glu122->Lys | Theophilus et al (2001) | Aug-01 | |
| 12 | 1866 | 603 | CTT CTC | Leu Leu | ? | ? | T/C | ? | - | Antonarakis et al (unpub) | Dec-91 | |
| 14 | 3376 | 1107 | AGG TGG | Arg Trp | <1 | ? | T/C | absent in at least 50 HA X chrs | also has IVS1 inversion | Jayandharan et al (2005) | Aug-07 | |
| 14 | 3376 | 1107 | AGG TGG | Arg Trp | <1 | ? | T/C | absent in at least 50 HA X chrs | also has R1966Stop | Jayandharan et al (2005) | Aug-07 | |
| 14 | 3780 | 1241 | GAC GAG | Asp Glu | ? | ? | C/G | ?; 0.73/0.27; 0.988/0.012* | - | Gitschier et al (1984); Higuchi et al (1991b); Lin et al (1993) | Dec-91 | |
| 14 | 3780 | 1241 | GAC GAG | Asp Glu | ? | ? | C>G | ? | mild, no other mutation found | Klopp et al (2002) | Dec-02 | |
| 14 | 3780 | 1241 | GAC GAG | Asp Glu | ? | ? | C>G | ? | severe, no other mutation found | Klopp et al (2002) | Dec-02 | |
| 14 | 3780 | 1241 | GAC GAG | Asp Glu | ? | ? | C>G | ? | severe, also has Y813X | Klopp et al (2002) | Dec-02 | |
| 14 | 3780 | 1241 | GAC GAG | Asp Glu | ? | ? | C>G | ? | severe, also has IVS22 inversion | Becker et al (1996) | Aug-96 | |
| 14 | 3780 | 1241 | GAC GAG | Asp Glu | ? | ? | C>G | ? | moderate, no other mutation found | Fernandez-Lopez et al (2005) | Jul-07 | |
| 14 | 3780 | 1241 | GAC GAG | Asp Glu | ? | ? | C>G | ? | moderate, no other mutation found | Fernandez-Lopez et al (2005) | Jul-07 | |
| 14 | 3780 | 1241 | GAC GAG | Asp Glu | 2 | ? | C>G | ? | moderate, no other mutation found | Jayandharan et al (2005) | Aug-07 | |
| 14 | 3864 | 1269 | TCA TCC | Ser Ser | ? | ? | A/C | 0.937/0.063; 0.82/0.18* | - | Higuchi et al (1991b); Lin et al (1993) | Dec-91 | |
| 14 | 3864 | 1269 | TCA TCC | Ser Ser | ? | ? | A>C | ? | severe, also has K946X | Klopp et al (2002) | Dec-02 | |
| 14 | 3864 | 1269 | TCA TCC | Ser Ser | ? | ? | A>C | ? | severe, also has frameshift ins | Klopp et al (2002) | Dec-02 | |
| 14 | 3864 | 1269 | TCA TCC | Ser Ser | ? | ? | A>C | ? | severe, also has frameshift ins | Klopp et al (2002) | Dec-02 | |
| 14 | 3864 | 1269 | TCA TCC | Ser Ser | ? | ? | A>C | ? | severe, also has frameshift ins | Klopp et al (2002) | Dec-02 | |
| 14 | 3931 | 1288 | GTA GTC | Val Val | ? | ? | A/C | 0.937/0.063 | - | Higuchi et al (1991b) | Dec-91 | |
| 14 | 4440 | 1462 | CTG CCG | Leu Pro | ? | ? | T/C | ? | - | Higuchi et al (1991a) | Dec-91 | |
| 14 | 4521 | 1488 | ACA ACC | Thr Thr | ? | ? | A/C | ? | - | Higuchi et al (1991a) | Dec-91 | |
| 17 | 4521 | 1882 | ATC ATA | Ile Ile | 8 | ? | C/A | ? | mild, no other mutation found | Jayandharan et al (2005) | Aug-07 | |
| 24 | 6642 | 2195 | TAC TAT | Tyr Tyr | ? | ? | C/T | ? | - | Antonarakis et al (unpub) | Dec-91 | |
| 25 | 6724 | 2223 | GTG ATG | Val Met | ? | ? | ? | ? | PM on normal X of carrier | Chan et al (1996) | Dec-96 | |
| 25 | 6724 | 2223 | GTG ATG | Val Met | ? | ? | G/A | 0.97/0.03; 0.02/0.98* (?error) | - | Antonarakis et al (unpub); Lin et al (1993) | Dec-93 | |
| 25 | 6724 | 2223 | GTG ATG | Val Met | 101 | ? | G/A | ? | - | Sommer et al (unpublished) | Dec-90 | |
| 25 | 6724 | 2223 | GTG ATG | Val Met | ? | ? | G/A | 0.98/0.02 | - | Gau et al (2006) | Jun-07 | |
| 25 | 6745 | 2230 | CTG TTG | Leu Leu | 6 | ? | C/T | ? | also has Phe679Leu | Timur et al (2001) | Oct-01 | |
| 25 | 6769 | 2238 | ATG GTG | Met Val | <2 | ? | A/G | 42% heterozygosity in Afro-Caribbean* | previously thought to be causative | Williams et al (1998) | Jul-98 | |
| N/A | 8728 | 110nt 5' poly A | 3' untrans | - | ? | ? | A/G | ?; 0.68/0.32 | - | Gitschier et al (1984); Higuchi et al (1991a) | Dec-91 | |
| N/A | 8728 | 110nt 5' poly A | 3' untrans | - | ? | ? | A/G | ? | mild, no other mutation found | Klopp et al (2002) | Dec-02 | |
| N/A | 8728 | 110nt 5' poly A | 3' untrans | - | ? | ? | A/G | ? | severe, no other mutation found | Klopp et al (2002) | Dec-02 | |
| N/A | 8728 | 110nt 5' poly A | 3' untrans | - | ? | ? | A/G | ? | severe, also has Y813X | Klopp et al (2002) | Dec-02 | |
| N/A | 8728 | 110nt 5' poly A | 3' untrans | - | ? | ? | A/G | ? | severe, also has K946X | Klopp et al (2002) | Dec-02 | |
| N/A | 8728 | 110nt 5' poly A | 3' untrans | - | ? | ? | A/G | ? | severe, also has frameshift ins | Klopp et al (2002) | Dec-02 | |
| N/A | 8728 | 110nt 5' poly A | 3' untrans | - | ? | ? | A/G | ? | severe, also has frameshift ins | Klopp et al (2002) | Dec-02 | |