| Mutation type:Point | Mutation effect:Missense | Nucleotide number:202 | |||
| Genome location:Exon3 | Subdomain:Gla | ||||
| Alpha missense prediction value:0.5914 | Alpha missense prediction class:pathogenic | ||||
| No. of patients reported: 3 | |||||
| Patient information: Monoallelic variation (3); 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 | F | NA | hetero | 81 | 45 | 117 | II | asymptomatic | NA | Japan | NA | PMID: 7482420; PMID: 8115990 |
| 2 | M | NA | hetero | 62 | 35 | 70 | II | asymptomatic | NA | Japan | NA | PMID: 7482420; PMID: 8115990 |
| 3 | M | NA | hetero | 84 | 52 | 97 | II | asymptomatic | NA | Japan | NA | PMID: 7482420; PMID: 8115990 |
| Mutation type:Point | Mutation effect:Missense | Nucleotide number:203 | |||
| Genome location:Exon3 | Subdomain:Gla | ||||
| Alpha missense prediction value:0.6932 | Alpha missense prediction class:pathogenic | ||||
| No. of patients reported: 2 | |||||
| Patient information: Monoallelic variation (2); 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 | 74 | 43.5 | 67 | IIb | Other VTE | NA | France | NA | PMID: 32717757 |
| 2 | NA | NA | hetero | 74 | 43.5 | 67 | IIb | Other VTE | NA | France | NA | PMID: 32717757 |
| Mutation type:Point; Point | Mutation effect:Missense; Missense | Nucleotide number:142; 202 | |||
| Genome location:Exon3; Exon3 | Subdomain:Gla; Gla | ||||
| Alpha missense prediction value:0.6171; 0.5914 | Alpha missense prediction class:pathogenic; 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 | M | NA | c.hetero | NA | <5 | NA | NA | BD, PF | 0-1y | Japan | ICTH (BD) | PMID: 28111891 |
| Mutation type:Point; Point | Mutation effect:Missense; Missense | Nucleotide number:202; 1015 | |||
| Genome location:Exon3; Exon9 | Subdomain:Gla; Serine protease | ||||
| Alpha missense prediction value:0.5914; 0.8511 | Alpha missense prediction class:pathogenic; 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 | NA | c.hetero | NA | 17 | NA | I | BD, PF (4D) | 4D | Japan | ICTH (BD) | PMID: 28111891 |
| Mutation type:Point; Deletion | Mutation effect:Missense; Frameshift | Nucleotide number:202; 1268 | |||
| Genome location:Exon3; Exon9 | Subdomain:Gla; Serine protease | ||||
| Alpha missense prediction value:0.5914 | 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 | M | 0Y | c.hetero | 12.5 | NA | 20 | I | PF, BD, DIC | 0Y | Japan | intracranial bleeding (BD); anticoagulation therapy | PMID: 7482420; PMID: 8115990 |