Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
St. |
RCV001543127 | SCV001761646 | uncertain significance | Ataxia-pancytopenia syndrome | 2021-07-26 | criteria provided, single submitter | clinical testing | The SAMD9L c.3483A>T (p.Arg1161Ser) missense change is absent in gnomAD v2.1.1 (PM2_supporting; https://gnomad.broadinstitute.org/). Seven of seven in silico tools predict a benign effect of this variant on protein function (BP4), but to our knowledge these predictions have not been confirmed by functional assays. To our knowledge, this variant has not been reported in individuals with ataxia-pancytopenia syndrome. In summary, this variant meets criteria to be classified as of uncertain significance based on the ACMG/AMP criteria: PM2_supporting, BP4. |
Prevention |
RCV004536167 | SCV004120950 | uncertain significance | SAMD9L-related disorder | 2023-02-28 | criteria provided, single submitter | clinical testing | The SAMD9L c.3483A>T variant is predicted to result in the amino acid substitution p.Arg1161Ser. To our knowledge, this variant has not been reported in the literature or in a large population database (http://gnomad.broadinstitute.org), indicating this variant is rare. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |
Ambry Genetics | RCV004952974 | SCV005499865 | uncertain significance | Inborn genetic diseases | 2024-12-06 | criteria provided, single submitter | clinical testing | The p.R1161S variant (also known as c.3483A>T), located in coding exon 1 of the SAMD9L gene, results from an A to T substitution at nucleotide position 3483. The arginine at codon 1161 is replaced by serine, an amino acid with dissimilar properties. This amino acid position is conserved. In addition, this alteration is predicted to be tolerated by in silico analysis. Based on the available evidence, the clinical significance of this variant remains unclear. |