Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000523671 | SCV000618726 | uncertain significance | not provided | 2023-09-13 | criteria provided, single submitter | clinical testing | 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: 35295078) |
Baylor Genetics | RCV001332577 | SCV001524947 | uncertain significance | Autosomal recessive severe congenital neutropenia due to CSF3R deficiency | 2020-05-05 | criteria provided, single submitter | clinical testing | This variant was determined to be of uncertain significance according to ACMG Guidelines, 2015 [PMID:25741868]. |
Labcorp Genetics |
RCV001332577 | SCV004531298 | uncertain significance | Autosomal recessive severe congenital neutropenia due to CSF3R deficiency | 2024-07-02 | criteria provided, single submitter | clinical testing | This sequence change replaces glycine, which is neutral and non-polar, with arginine, which is basic and polar, at codon 27 of the CSF3R protein (p.Gly27Arg). This variant is present in population databases (rs759364352, gnomAD 0.003%). This missense change has been observed in individual(s) with severe congenital neutropenia (PMID: 35295078). ClinVar contains an entry for this variant (Variation ID: 450176). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt CSF3R protein function with a positive predictive value of 80%. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
Genomic Medicine Center of Excellence, |
RCV001332577 | SCV005441880 | uncertain significance | Autosomal recessive severe congenital neutropenia due to CSF3R deficiency | 2024-12-19 | criteria provided, single submitter | clinical testing |