Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001052861 | SCV001217093 | likely pathogenic | Multiple gastrointestinal atresias | 2019-02-26 | criteria provided, single submitter | clinical testing | In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. Loss-of-function variants in TTC7A are known to be pathogenic (PMID: 23830146, 24292712). This variant has not been reported in the literature in individuals with TTC7A-related conditions. The frequency data for this variant in the population databases is considered unreliable, as metrics indicate insufficient coverage at this position in the ExAC database. This variant is a deletion of the genomic region encompassing the consensus acceptor splice site in intron 3 and part of exon 4 (c.518-914_588del) of the TTC7A gene. It is expected to disrupt RNA splicing and likely results in an absent or disrupted protein product. |