Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001776380 | SCV002012901 | uncertain significance | not provided | 2019-07-12 | criteria provided, single submitter | clinical testing | Has not been previously published as pathogenic or benign to our knowledge; Not observed at a significant frequency in large population cohorts (Lek et al., 2016); In silico analysis, which includes protein predictors and evolutionary conservation, supports that this variant does not alter protein structure/function |
Labcorp Genetics |
RCV001885129 | SCV002298250 | uncertain significance | Ullrich congenital muscular dystrophy 2; Bethlem myopathy 2 | 2025-01-28 | criteria provided, single submitter | clinical testing | This sequence change replaces aspartic acid, which is acidic and polar, with glycine, which is neutral and non-polar, at codon 1921 of the COL12A1 protein (p.Asp1921Gly). This variant is present in population databases (rs757348453, gnomAD 0.006%). This variant has not been reported in the literature in individuals affected with COL12A1-related conditions. ClinVar contains an entry for this variant (Variation ID: 1320401). 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 COL12A1 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. |
Center for Genomics, |
RCV001885129 | SCV002496061 | uncertain significance | Ullrich congenital muscular dystrophy 2; Bethlem myopathy 2 | 2021-10-21 | criteria provided, single submitter | clinical testing | COL12A1 NM_004370.5 exon 34 p.Asp1921Gly (c.5762A>G): This variant has not been reported in the literature but is present in 0.03% (6/15270) of European alleles in the Genome Aggregation Database (https://gnomad.broadinstitute.org/variant/6-75133325-T-C?dataset=gnomad_r3). Evolutionary conservation suggests that this variant may not impact the protein; computational predictive tools for this variant are unclear. In summary, data on this variant is insufficient for disease classification. Therefore, the clinical significance of this variant is uncertain. |
Revvity Omics, |
RCV001776380 | SCV003833402 | uncertain significance | not provided | 2019-08-05 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV004611874 | SCV005108849 | uncertain significance | Inborn genetic diseases | 2024-06-07 | criteria provided, single submitter | clinical testing | The c.5762A>G (p.D1921G) alteration is located in exon 34 (coding exon 33) of the COL12A1 gene. This alteration results from a A to G substitution at nucleotide position 5762, causing the aspartic acid (D) at amino acid position 1921 to be replaced by a glycine (G). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |