Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002975501 | SCV003282765 | uncertain significance | Autosomal recessive ataxia, Beauce type; Emery-Dreifuss muscular dystrophy 4, autosomal dominant | 2022-05-23 | 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 (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be tolerated. This sequence change replaces leucine, which is neutral and non-polar, with phenylalanine, which is neutral and non-polar, at codon 3869 of the SYNE1 protein (p.Leu3869Phe). This variant is present in population databases (rs779176432, gnomAD 0.003%). This variant has not been reported in the literature in individuals affected with SYNE1-related conditions. |
Ambry Genetics | RCV002975502 | SCV003540300 | uncertain significance | Inborn genetic diseases | 2021-08-30 | criteria provided, single submitter | clinical testing | The c.11607G>C (p.L3869F) alteration is located in exon 71 (coding exon 70) of the SYNE1 gene. This alteration results from a G to C substitution at nucleotide position 11607, causing the leucine (L) at amino acid position 3869 to be replaced by a phenylalanine (F). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |