Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Baylor Genetics | RCV001333744 | SCV001526418 | uncertain significance | Atypical glycine encephalopathy | 2018-12-22 | criteria provided, single submitter | clinical testing | This variant was determined to be of uncertain significance according to ACMG Guidelines, 2015 [PMID:25741868]. |
Labcorp Genetics |
RCV001333744 | SCV003295227 | uncertain significance | Atypical glycine encephalopathy | 2023-04-24 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. Variants that disrupt the consensus splice site are a relatively common cause of aberrant splicing (PMID: 17576681, 9536098). 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: 1031818). This variant has not been reported in the literature in individuals affected with SLC6A9-related conditions. This variant is present in population databases (rs201870833, gnomAD 0.002%). This sequence change affects codon 394 of the SLC6A9 mRNA. It is a 'silent' change, meaning that it does not change the encoded amino acid sequence of the SLC6A9 protein. This variant also falls at the last nucleotide of exon 8, which is part of the consensus splice site for this exon. |