Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003009211 | SCV003298435 | uncertain significance | Emery-Dreifuss muscular dystrophy 5, autosomal dominant | 2022-12-15 | criteria provided, single submitter | clinical testing | 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. Algorithms developed to predict the effect of missense changes on protein structure and function output the following: SIFT: "Tolerated"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C0". The phenylalanine amino acid residue is found in multiple mammalian species, which suggests that this missense change does not adversely affect protein function. This variant has not been reported in the literature in individuals affected with SYNE2-related conditions. This variant is not present in population databases (gnomAD no frequency). This sequence change replaces leucine, which is neutral and non-polar, with phenylalanine, which is neutral and non-polar, at codon 1884 of the SYNE2 protein (p.Leu1884Phe). |
Prevention |
RCV003404001 | SCV004105468 | uncertain significance | SYNE2-related disorder | 2022-10-04 | criteria provided, single submitter | clinical testing | The SYNE2 c.5650C>T variant is predicted to result in the amino acid substitution p.Leu1884Phe. 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 | RCV004673758 | SCV005165885 | uncertain significance | not specified | 2024-05-14 | criteria provided, single submitter | clinical testing | The c.5650C>T (p.L1884F) alteration is located in exon 39 (coding exon 38) of the SYNE2 gene. This alteration results from a C to T substitution at nucleotide position 5650, causing the leucine (L) at amino acid position 1884 to be replaced by a phenylalanine (F). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |