Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Departamento de Patología, |
RCV001528189 | SCV001739515 | likely pathogenic | Hereditary factor VIII deficiency disease | criteria provided, single submitter | clinical testing | ACMG Guidelines. PM1: UniProt protein FA8_HUMAN domain 'Plastocyanin-like 6' has 55 non-VUS missense/in-frame/non-synonymous, variants (55 pathogenic and 0 benign), pathogenicity = 100.0% which is more than threshold 50.0%. PM2: Variant not found in gnomAD exomes (good gnomAD exomes coverage = 83.8). Variant not found in gnomAD genomes (good gnomAD genomes coverage = 22.0). PP2: 720 out of 744 non-VUS. PP3: missense variants in gene F8 are pathogenic = 96.8% which is more than threshold of 51.0%, and 856 out of 996 clinically reported variants in gene F8 are pathogenic = 85.9% which is more than threshold of 12.0%. PP3: Pathogenic computational verdict based on 7 pathogenic predictions from DANN, DEOGEN2, FATHMM-MKL, M-CAP, MVP, MutationAssessor and MutationTaster vs 4 benign predictions from BayesDel_addAF, LIST-S2, PrimateAI and SIFT. |