Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
New York Genome Center | RCV002468184 | SCV002764410 | uncertain significance | Hypercholesterolemia, autosomal dominant, type B; Familial hypobetalipoproteinemia 1 | 2020-11-18 | criteria provided, single submitter | clinical testing | The c.7118C>T (p.Thr2373Ile) variant identified in the APOB gene substitutes a moderately conserved Threonine for Isoleucine at amino acid 2373/4564 (exon 26/29). This variant is absent from gnomAD(v3.0) suggesting it is not a common benign variant in the populations represented in that database. In silico algorithms do not agree on the effect of this variant, as it is predicted both Damaging (SIFT; score:0.024) and Benign (REVEL; score:0.094) to the function of the canonical transcript. This variant is reported in ClinVar (VarID:925059) as a Variant of Uncertain Significance, and to our current knowledge has not been reported in affected individuals in the literature. Given the lack of compelling evidence for its pathogenicity, the c.7118C>T (p.Thr2373Ile) variant identified in the APOB gene is reported as a Variant of Uncertain Significance. |