Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000723545 | SCV000110147 | pathogenic | not provided | 2012-08-15 | criteria provided, single submitter | clinical testing | |
Invitae | RCV000011488 | SCV002158551 | pathogenic | Fabry disease | 2021-01-31 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. This variant disrupts the p.Gly328 amino acid residue in GLA. Other variant(s) that disrupt this residue have been observed in individuals with GLA-related conditions (PMID: 16595074, 15712228, 1315715), which suggests that this may be a clinically significant amino acid residue. Experimental studies have shown that this variant affects GLA protein function (PMID: 21598360, 23935525). This variant has been observed in individual(s) with Fabry disease (PMID: 27834756, 16595074, 7504405). ClinVar contains an entry for this variant (Variation ID: 10740). This variant is not present in population databases (ExAC no frequency). This sequence change replaces glycine with alanine at codon 328 of the GLA protein (p.Gly328Ala). The glycine residue is highly conserved and there is a small physicochemical difference between glycine and alanine. |
OMIM | RCV000011488 | SCV000031720 | pathogenic | Fabry disease | 1993-12-01 | no assertion criteria provided | literature only |