Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000231902 | SCV000290166 | uncertain significance | Familial aplasia of the vermis; Meckel-Gruber syndrome | 2023-11-28 | criteria provided, single submitter | clinical testing | This sequence change replaces glycine, which is neutral and non-polar, with serine, which is neutral and polar, at codon 37 of the B9D1 protein (p.Gly37Ser). This variant is present in population databases (rs771997194, gnomAD 0.01%). This missense change has been observed in individual(s) with clinical features of B9D1-related conditions (Invitae). ClinVar contains an entry for this variant (Variation ID: 241064). 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. 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. |
Prevention |
RCV004555547 | SCV004118078 | uncertain significance | B9D1-related disorder | 2023-05-24 | criteria provided, single submitter | clinical testing | The B9D1 c.109G>A variant is predicted to result in the amino acid substitution p.Gly37Ser. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.014% of alleles in individuals of Latino descent in gnomAD (http://gnomad.broadinstitute.org/variant/17-19263656-C-T). At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |