Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Rady Children's Institute for Genomic Medicine, |
RCV001267661 | SCV001445884 | likely pathogenic | Congenital microvillous atrophy | 2019-05-02 | criteria provided, single submitter | clinical testing | A heterozygous c.1303G>A (p.Gly435Arg) variant and a heterozygous c.947G>T (p.Gly316Val) variant in the MYO5B gene were detected in this individual. Analysis of the parental samples indicated these variants are in trans configuration (i.e. compound heterozygous). The c.1303G>A (p.Gly435Arg) variant has been previously reported as a heterozygous change in a patient with microvillus inclusion disease (PMID: 20186687). It is absent from the ExAC and gnomAD population databases and thus is presumed to be rare. The c.1303G>A variant affects a highly conserved amino acid and is predicted by multiple in silico tools to have a deleterious effect on protein function. Based on the available evidence, the c.1303G>A (p.Gly435Arg) variant is classified as Likely Pathogenic. |
Aleixo Muise Laboratory, |
RCV001267661 | SCV005088095 | pathogenic | Congenital microvillous atrophy | 2024-07-05 | criteria provided, single submitter | research | PS1;PM2;PM3;PP3;PP4 |