Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Reproductive Genetics, |
RCV000791328 | SCV000882699 | likely pathogenic | Non-obstructive azoospermia; Spermatogenesis maturation arrest | 2019-01-31 | criteria provided, single submitter | research | The index patient with azoospermia due to meiotic arrest carries two rare variants in exon 13 of the STAG3 gene (c.1262T>G, p.Leu421Arg and c.1312C>T, p.Arg428Ter) in a compound heterozygous state (proven by analyses of the parents). The unaffected brother only carries the variant c.1262T>G. In silico prediction and in vitro experiments support pathogenicity of the compound heterozygous variants in this case (detailed in the manuscript). According to the 2015 ACMG guidelines, both variants are classified as likely pathogenic. |
OMIM | RCV001797138 | SCV002039150 | pathogenic | Spermatogenic failure 61 | 2021-12-20 | no assertion criteria provided | literature only |