Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV004700214 | SCV005201768 | likely pathogenic | not provided | 2023-09-22 | criteria provided, single submitter | clinical testing | Published functional studies demonstrate reduced F8 abundance and activity, as well as significantly reduced VWF binding (van den Biggelaar et al., 2011); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Not observed at significant frequency in large population cohorts (gnomAD); Also known as p.P2300S; This variant is associated with the following publications: (PMID: 19473423, 1908817, 9829908, 21909383, 36998198, 15471879) |
OMIM | RCV000010860 | SCV000031087 | pathogenic | Hereditary factor VIII deficiency disease | 1991-08-01 | no assertion criteria provided | literature only |