Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV002724776 | SCV003736424 | uncertain significance | Inborn genetic diseases | 2021-11-12 | criteria provided, single submitter | clinical testing | The c.3021T>G (p.D1007E) alteration is located in exon 23 (coding exon 22) of the CARD11 gene. This alteration results from a T to G substitution at nucleotide position 3021, causing the aspartic acid (D) at amino acid position 1007 to be replaced by a glutamic acid (E). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |
Labcorp Genetics |
RCV003778596 | SCV004596813 | uncertain significance | Severe combined immunodeficiency due to CARD11 deficiency; BENTA disease | 2023-03-14 | criteria provided, single submitter | clinical testing | This variant is present in population databases (rs780981181, gnomAD 0.02%). 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. An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be disruptive. ClinVar contains an entry for this variant (Variation ID: 2394813). This variant has not been reported in the literature in individuals affected with CARD11-related conditions. This sequence change replaces aspartic acid, which is acidic and polar, with glutamic acid, which is acidic and polar, at codon 1007 of the CARD11 protein (p.Asp1007Glu). |