Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001300917 | SCV001490068 | likely benign | Heterotopia, periventricular, X-linked dominant; Melnick-Needles syndrome; Oto-palato-digital syndrome, type II; Frontometaphyseal dysplasia | 2024-11-24 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV004036185 | SCV005017595 | uncertain significance | Familial thoracic aortic aneurysm and aortic dissection | 2024-02-02 | criteria provided, single submitter | clinical testing | The p.R7G variant (also known as c.19C>G), located in coding exon 1 of the FLNA gene, results from a C to G substitution at nucleotide position 19. The arginine at codon 7 is replaced by glycine, an amino acid with dissimilar properties. Based on data from gnomAD, the G allele has an overall frequency of <0.01% (3/171906) total alleles studied, with 1 hemizygote(s) observed. The highest observed frequency was <0.01% (3/74203) of European (non-Finnish) alleles. This amino acid position is well conserved in available vertebrate species. In addition, this alteration is predicted to be tolerated by in silico analysis. Based on the available evidence, the clinical significance of this alteration remains unclear. |