Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000041674 | SCV000065370 | uncertain significance | not specified | 2009-08-03 | criteria provided, single submitter | clinical testing | |
Counsyl | RCV000667791 | SCV000792295 | uncertain significance | Usher syndrome type 2A; Retinitis pigmentosa 39 | 2017-06-13 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001852852 | SCV002220064 | pathogenic | not provided | 2023-08-30 | criteria provided, single submitter | clinical testing | Variants that disrupt the consensus splice site are a relatively common cause of aberrant splicing (PMID: 17576681, 9536098). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be disruptive. ClinVar contains an entry for this variant (Variation ID: 48353). This variant has been observed in individuals with USH2A-related conditions (PMID: 20507924, 22135276, 25425308; Invitae). This variant is not present in population databases (gnomAD no frequency). This sequence change affects codon 3529 of the USH2A mRNA. It is a 'silent' change, meaning that it does not change the encoded amino acid sequence of the USH2A protein. This variant also falls at the last nucleotide of exon 53, which is part of the consensus splice site for this exon. For these reasons, this variant has been classified as Pathogenic. |
Baylor Genetics | RCV003466892 | SCV004208392 | likely pathogenic | Retinitis pigmentosa 39 | 2024-03-12 | criteria provided, single submitter | clinical testing |