Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV003991445 | SCV004809053 | uncertain significance | Creatine transporter deficiency | 2024-03-28 | reviewed by expert panel | curation | The NM_005629.4:c.1255-35G>A variant in SLC6A8 is an intronic variant affecting a nucleotide within the consensus splice site of intron 8. This variant has been reported as a novel variant in one individual undergoing genetic testing for suspected X-linked intellectual disability (PMID: 15154114), without further specification; thus PP4 does not apply. The variant is absent in gnomAD v2.1.1. (PM2_Supporting). The computational predictors SpliceAI and varSEAK predict that this variant does not impact splicing (BP4). In summary, this variant meets criteria to be classified as a variant of uncertain significance for creatine transporter deficiency. SLC6A8-specific criteria applied, as specified by the ClinGen CCDS VCEP (Specifications Version 1.1.0): PM2_Supporting, BP4. Curation Expert Panel on March 28, 2024) |