Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV004783723 | SCV005396197 | likely pathogenic | not provided | 2024-05-08 | criteria provided, single submitter | clinical testing | Published functional studies demonstrate that Q96P shows delayed activation by Factor XIa and reduced activity compared to wild-type Factor IX (PMID: 2306516); Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; This variant is associated with the following publications: (PMID: 22639855, 31253089, 2306516) |
OMIM | RCV000011323 | SCV000031554 | pathogenic | Hereditary factor IX deficiency disease | 2012-11-12 | no assertion criteria provided | literature only |