Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Revvity Omics, |
RCV001782311 | SCV002016732 | likely pathogenic | Congenital muscular dystrophy due to integrin alpha-7 deficiency | 2020-01-31 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001782311 | SCV002146753 | pathogenic | Congenital muscular dystrophy due to integrin alpha-7 deficiency | 2024-05-13 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg569*) in the ITGA7 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in ITGA7 are known to be pathogenic (PMID: 9590299). This variant is present in population databases (rs201217202, gnomAD 0.005%). This variant has not been reported in the literature in individuals affected with ITGA7-related conditions. ClinVar contains an entry for this variant (Variation ID: 1324591). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. For these reasons, this variant has been classified as Pathogenic. |