| Mutation type:Point | Mutation effect:Missense | Nucleotide number:541 | |||
| Genome location:Exon7 | Subdomain:Linker | ||||
| Alpha missense prediction value:0.4146 | Alpha missense prediction class:ambiguous | ||||
| No. of patients reported: 31 | |||||
| Patient information: Monoallelic variation (31); Biallelic variation (0) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | NA | NA | hetero | 59 | 65 | 61 | I | AMI (45), pulmonary atresia (45) | 45 | Japan | NA | PMID: 9840027 |
| 2 | NA | NA | hetero | 49 | 45 | 59 | I | NA | NA | Japan | NA | PMID: 9840027 |
| 3 | NA | NA | hetero | 49 | 41 | 60 | I | epilepsy | NA | Japan | NA | PMID: 9840027 |
| 4 | NA | NA | hetero | 62 | 60 | 62 | I | stroke (69) | 69 | Japan | NA | PMID: 8883262 |
| 5 | NA | NA | hetero | 63 | 35 | 62 | I | angina pectoris (28) | 28 | Japan | NA | PMID: 8883262 |
| 6 | NA | NA | hetero | 66 | NA | 61 | I | asymptomatic | NA | Japan | NA | PMID: 8883262 |
| 7 | M | 10 | hetero | NA | NA | NA | I | DVT (10) | 10 | China | NA | PMID: 37950050 |
| 8 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 37950050 |
| 9 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 37950050 |
| 10 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 37950050 |
| 11 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 37950050 |
| 12 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 37950050 |
| 13 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 37950050 |
| 14 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 37950050 |
| 15 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 37950050 |
| 16 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 37950050 |
| 17 | F | NA | hetero | NA | NA | NA | I | NA | NA | China | NA | PMID: 37789321 |
| 18 | M | NA | hetero | NA | NA | NA | I | NA | NA | China | NA | PMID: 37789321 |
| 19 | M | NA | hetero | NA | NA | NA | I | asymptomatic | NA | China | NA | PMID: 24911457 |
| 20 | F | 69 | hetero | 54 | NA | 47.1 | I | asymptomatic | 69 | China | NA | PMID: 24911457 |
| 21 | F | 51 | hetero | 60 | NA | 62.4 | I | asymptomatic | 51 | China | NA | PMID: 24911457 |
| 22 | F | 47 | hetero | 67 | NA | 60.3 | I | asymptomatic | 47 | China | NA | PMID: 24911457 |
| 23 | M | 49 | hetero | 58 | NA | 52.5 | I | asymptomatic | 49 | China | NA | PMID: 24911457 |
| 24 | F | 24 | hetero | 61 | NA | 65.8 | I | asymptomatic | 24 | China | NA | PMID: 24911457 |
| 25 | F | 25 | hetero | 51 | NA | 50.5 | I | asymptomatic | 25 | China | NA | PMID: 24911457 |
| 26 | M | 60 | hetero | 68 | NA | 71.2 | I | asymptomatic | 60 | China | NA | PMID: 24911457 |
| 27 | F | 51 | hetero | 74 | NA | 81.15 | I | asymptomatic | 51 | China | NA | PMID: 24911457 |
| 28 | F | 48 | hetero | 61 | NA | 59.75 | I | asymptomatic | 48 | China | NA | PMID: 24911457 |
| 29 | F | 88 | hetero | 66 | NA | 62.25 | I | asymptomatic | 88 | China | NA | PMID: 24911457 |
| 30 | NA | 64 | hetero | 44.2 | NA | 42.4 | I | DVT | 64 | China | NA | PMID: 22545135 |
| 31 | M | NA | hetero | 36.3 | NA | 38.09 | I | asymptomatic | NA | China | NA | PMID: 16689777 |
| Mutation type:Point | Mutation effect:Missense | Nucleotide number:541 | |||
| Genome location:Exon7 | Subdomain:Linker | ||||
| Alpha missense prediction value:0.9267 | Alpha missense prediction class:pathogenic | ||||
| No. of patients reported: 8 | |||||
| Patient information: Monoallelic variation (8); Biallelic variation (0) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | NA | NA | hetero | 56 | NA | 57 | I | Other VTE | NA | USA | thrombosis (Other VTE) | PMID: 8972002 |
| 2 | NA | NA | hetero | 55.5 | 70.5 | 53 | I | asymptomatic | NA | France | NA | PMID: 32717757 |
| 3 | NA | NA | hetero | 55.5 | 70.5 | 53 | I | asymptomatic | NA | France | NA | PMID: 32717757 |
| 4 | F | NA | hetero | 64 | NA | NA | I | asymptomatic | NA | Australia | NA | PMID: 26103879 |
| 5 | M | 8 | hetero | 56 | NA | NA | I | asymptomatic | 8 | Australia | NA | PMID: 26103879 |
| 6 | NA | NA | hetero | 80 | NA | 60 | I | NA | NA | UK | NA | PMID: 7482420 |
| 7 | NA | NA | hetero | 56 | NA | 67 | I | NA | NA | UK | NA | PMID: 7482420 |
| 8 | NA | NA | hetero | NA | NA | NA | I | asymptomatic | NA | UK | NA | PMID: 7482420 |
| Mutation type:Point; Point | Mutation effect:Missense; Missense | Nucleotide number:541; 541 | |||
| Genome location:Exon7; Exon7 | Subdomain:Linker; Linker | ||||
| Alpha missense prediction value:0.4146; 0.4146 | Alpha missense prediction class:ambiguous; ambiguous | ||||
| No. of patients reported: 4 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (4) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 43 | homo | 20 | NA | 13 | I | experienced pain and restricted joint movement (8); DVT (43) | 8 | China | NA | PMID: 37950050 |
| 2 | F | 43 | homo | 20 | NA | 13 | I | DVT (43) | 43 | China | consanguineous marriage; Long-term use of oral contraceptives | PMID: 35231991 |
| 3 | F | 43 | homo | 20 | NA | 13.2 | I | DVT (43), arthralgia in four limbs (8), hypertension and hepatitis | 8 | China | Salvia miltiorrhiza and ibuprofen. anticoagulation therapy. consanguineous marriage. Oral contraceptive | PMID: 24911457 |
| 4 | M | NA | homo | 25.67±5.51 | NA | 25.67±5.51 | I | DVT (10), arthralgia in four limbs (10), hypertension and hepatitis | 10 | China | consanguineous marriage | PMID: 24911457 |
| Mutation type:Point; Point | Mutation effect:Missense; Missense | Nucleotide number:318; 541 | |||
| Genome location:Exon5; Exon7 | Subdomain:EGF1; Linker | ||||
| Alpha missense prediction value:0.9044; 0.4146 | Alpha missense prediction class:pathogenic; ambiguous | ||||
| No. of patients reported: 1 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (1) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 32 | c.hetero | 1.2 | NA | 1 | I | DVT (24, 30), Other VTE (30) | 24 | China | thrombosis (Other VTE) | PMID: 16689777 |
| Mutation type:Deletion; Point | Mutation effect:Frameshift; Missense | Nucleotide number:349; 541 | |||
| Genome location:Exon5; Exon7 | Subdomain:EGF1; Linker | ||||
| Alpha missense prediction value:0.4146 | Alpha missense prediction class:ambiguous | ||||
| No. of patients reported: 1 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (1) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | NA | 24 | c.hetero | 9.9 | NA | 9.2 | I | DVT, PE | 24 | China | NA | PMID: 22545135 |
| Mutation type:Deletion; Point | Mutation effect:Frameshift; Missense | Nucleotide number:388; 541 | |||
| Genome location:Exon5; Exon7 | Subdomain:EGF1; Linker | ||||
| Alpha missense prediction value:0.9267 | Alpha missense prediction class:pathogenic | ||||
| No. of patients reported: 1 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (1) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 18 | c.hetero | 3-13 | NA | NA | I | multiple DVT (18, recurrent), PE, splenic infarction | 18 | Australia | LMWH, protein C concentrate, anticoagulation therapy, rivaroxaban, below-knee amputation. | PMID: 26103879 |
| Mutation type:Point; Point | Mutation effect:Missense; Missense | Nucleotide number:541; 565 | |||
| Genome location:Exon7; Exon7 | Subdomain:Linker; Linker | ||||
| Alpha missense prediction value:0.4146; 0.0952 | Alpha missense prediction class:ambiguous; benign | ||||
| No. of patients reported: 1 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (1) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 24 | c.hetero | NA | NA | PC defect | I | DVT (14, 22, 23), APS | 14 | China | NA | PMID: 37789321 |
| Mutation type:Point; Deletion | Mutation effect:Missense; Inframe | Nucleotide number:541; 574 | |||
| Genome location:Exon7; Exon7 | Subdomain:Linker; Linker | ||||
| Alpha missense prediction value:0.4146 | Alpha missense prediction class:ambiguous | ||||
| No. of patients reported: 4 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (4) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 28 | c.hetero | 23 | NA | 57 | I | PE (28) | 28 | China | NA | PMID: 37950050 |
| 2 | M | 34 | c.hetero | NA | NA | PC defect | I | PE (33) | 33 | China | NA | PMID: 37789321 |
| 3 | M | 28 | c.hetero | 23 | NA | 57 | I | PE (28) | 28 | China | NA | PMID: 35231991 |
| 4 | F | NA | c.hetero | 36.5 | NA | 39.71 | I | asymptomatic | NA | China | NA | PMID: 16689777 |
| Mutation type:Point; Point | Mutation effect:Missense; Nonsense | Nucleotide number:541; 595 | |||
| Genome location:Exon7; Exon7 | Subdomain:Linker; Linker | ||||
| Alpha missense prediction value:0.4146 | Alpha missense prediction class:ambiguous | ||||
| No. of patients reported: 1 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (1) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | NA | NA | c.hetero | 19.1 | NA | NA | I | DVT | NA | China | NA | PMID: 24028705 |
| Mutation type:Point; Point | Mutation effect:Missense; Missense | Nucleotide number:541; 889 | |||
| Genome location:Exon7; Exon9 | Subdomain:Linker; Serine protease | ||||
| Alpha missense prediction value:0.4146; 0.735 | Alpha missense prediction class:ambiguous; pathogenic | ||||
| No. of patients reported: 3 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (3) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 30 | c.hetero | 26 | NA | 19 | I | Other VTE (30) | 30 | China | CVT (Other VTE) | PMID: 37950050 |
| 2 | F | 30 | c.hetero | 26 | NA | 19 | I | Other VTE (30) | 30 | China | CVT (Other VTE); CI and DVT in great-uncle and second-uncle, respectively. | PMID: 35231991 |
| 3 | F | 30 | c.hetero | 26 | NA | 18.6 | I | Other VTE (30), BD | 30 | China | thrombosis (Other VTE), ICTH (BD), hemorrhage (BD); heparin, anticoagulation therapy | PMID: 24911457 |
| Mutation type:Point; Point | Mutation effect:Missense; Missense | Nucleotide number:541; 926 | |||
| Genome location:Exon7; Exon9 | Subdomain:Linker; Serine protease | ||||
| Alpha missense prediction value:0.4146; 0.1007 | Alpha missense prediction class:ambiguous; benign | ||||
| No. of patients reported: 1 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (1) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 12 | c.hetero | NA | 27 | 21 | I | Epilepsy (3), Other VTE (12) | 3 | Japan | thrombosis (Other VTE) | PMID: 28111891 |
| Mutation type:Point; Point | Mutation effect:Missense; Missense | Nucleotide number:541; 970 | |||
| Genome location:Exon7; Exon9 | Subdomain:Linker; Serine protease | ||||
| Alpha missense prediction value:0.4146; 0.0801 | Alpha missense prediction class:ambiguous; benign | ||||
| No. of patients reported: 1 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (1) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | NA | NA | c.hetero | 47 | 50 | 45 | I | stroke (58) | 58 | Japan | NA | PMID: 8883262 |
| Mutation type:Point; Point | Mutation effect:Missense; Missense | Nucleotide number:541; 980 | |||
| Genome location:Exon7; Exon9 | Subdomain:Linker; Serine protease | ||||
| Alpha missense prediction value:0.4146; 0.3181 | Alpha missense prediction class:ambiguous; benign | ||||
| No. of patients reported: 1 | |||||
| Patient information: Monoallelic variation (0); Biallelic variation (1) | |||||
| Patient | Sex | Age | Genotype | PC: amidolytic act (%) | PC: clotting act (%) | PC: Ag (%) | PCD type | Clinical presentation (age) | First onset age | Country | Comments | Reference |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | NA | 42 | c.hetero | 39.4 | NA | 26.7 | I | DVT, acute ischemic stroke | 42 | China | NA | PMID: 22545135 |