Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000803979 | SCV000943868 | uncertain significance | Amelocerebrohypohidrotic syndrome | 2018-07-01 | 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. Nucleotide substitutions within 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 create or strengthen a splice site, but this prediction has not been confirmed by published transcriptional studies. This variant has not been reported in the literature in individuals with ROGDI-related disease. This variant is present in population databases (rs765836538, ExAC 0.01%). This sequence change falls in intron 5 of the ROGDI gene. It does not directly change the encoded amino acid sequence of the ROGDI protein, but it affects a nucleotide within the consensus splice site of the intron. |
Ambry Genetics | RCV003166234 | SCV003896041 | uncertain significance | Inborn genetic diseases | 2023-03-06 | criteria provided, single submitter | clinical testing | The c.336+3G>A intronic alteration consists of a G to A substitution 3 nucleotides after exon 5 of the ROGDI gene. Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |