Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV002509643 | SCV002817383 | likely pathogenic | Premature ovarian failure 7 | 2023-01-03 | criteria provided, single submitter | clinical testing | |
Invitae | RCV002537605 | SCV003026220 | uncertain significance | Oligosynaptic infertility; 46,XY disorder of sex development | 2022-05-15 | criteria provided, single submitter | clinical testing | This sequence change replaces valine, which is neutral and non-polar, with methionine, which is neutral and non-polar, at codon 355 of the NR5A1 protein (p.Val355Met). This variant is present in population databases (rs371701248, gnomAD 0.03%). This missense change has been observed in individual(s) with anorchia or idiopathic oligozoospermia (PMID: 17940071, 32655042). Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Possibly Damaging"; Align-GVGD: "Class C15"). Experimental studies are conflicting or provide insufficient evidence to determine the effect of this variant on NR5A1 function (PMID: 17940071, 30067310). 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. |
Institute of Reproductive Genetics, |
RCV003983865 | SCV004800827 | likely pathogenic | Male infertility | 2024-01-16 | criteria provided, single submitter | research |