Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001219488 | SCV001391431 | uncertain significance | Hereditary spastic paraplegia 3A | 2024-09-17 | criteria provided, single submitter | clinical testing | This sequence change replaces alanine, which is neutral and non-polar, with glycine, which is neutral and non-polar, at codon 229 of the ATL1 protein (p.Ala229Gly). This variant is present in population databases (no rsID available, gnomAD 0.002%). This variant has not been reported in the literature in individuals affected with ATL1-related conditions. ClinVar contains an entry for this variant (Variation ID: 948267). Invitae Evidence Modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) indicates that this missense variant is not expected to disrupt ATL1 protein function with a negative predictive value of 80%. 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. |
Athena Diagnostics | RCV001288451 | SCV001475556 | uncertain significance | not provided | 2020-03-04 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002365992 | SCV002665617 | uncertain significance | Inborn genetic diseases | 2019-09-18 | criteria provided, single submitter | clinical testing | The p.A229G variant (also known as c.686C>G), located in coding exon 7 of the ATL1 gene, results from a C to G substitution at nucleotide position 686. The alanine at codon 229 is replaced by glycine, an amino acid with similar properties. This amino acid position is poorly conserved in available vertebrate species. In addition, this alteration is predicted to be tolerated by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |