Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000533204 | SCV000655016 | likely pathogenic | Cataract 21 multiple types; Ayme-Gripp syndrome | 2018-01-12 | criteria provided, single submitter | clinical testing | This variant has been reported to be de novo in an individual affected with bilateral congenital cataracts (Invitae). In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. Algorithms developed to predict the effect of missense changes on protein structure and function (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be disruptive, but these predictions have not been confirmed by published functional studies and their clinical significance is uncertain. This variant is not present in population databases (ExAC no frequency). This sequence change replaces alanine with valine at codon 302 of the MAF protein (p.Ala302Val). The alanine residue is highly conserved and there is a small physicochemical difference between alanine and valine. |
Molecular Medicine, |
RCV003486884 | SCV004023263 | likely pathogenic | Cataract 21 multiple types | 2023-07-28 | criteria provided, single submitter | research |