Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001940010 | SCV002182120 | pathogenic | Autosomal recessive severe congenital neutropenia due to CSF3R deficiency | 2024-11-06 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg74Glnfs*7) in the CSF3R gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in CSF3R are known to be pathogenic (PMID: 24753537, 26324699). This variant is present in population databases (rs765536522, gnomAD 0.003%). This variant has not been reported in the literature in individuals affected with CSF3R-related conditions. ClinVar contains an entry for this variant (Variation ID: 1410136). For these reasons, this variant has been classified as Pathogenic. |
Prevention |
RCV003892946 | SCV004711826 | likely pathogenic | CSF3R-related disorder | 2023-12-04 | no assertion criteria provided | clinical testing | The CSF3R c.218dupG variant is predicted to result in a frameshift and premature protein termination (p.Arg74Glnfs*7). To our knowledge, this variant has not been reported in the literature. It is reported in 0.0065% of alleles in individuals of European (Non-Finnish) descent in gnomAD. Frameshift variants in CSF3R are expected to be pathogenic. This variant is interpreted as likely pathogenic. |