Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Invitae | RCV001034975 | SCV001198279 | likely benign | RYR1-related disorder | 2024-01-28 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV002489536 | SCV002790335 | uncertain significance | Central core myopathy; Malignant hyperthermia, susceptibility to, 1; Congenital multicore myopathy with external ophthalmoplegia; Congenital myopathy with fiber type disproportion; King Denborough syndrome | 2021-09-14 | criteria provided, single submitter | clinical testing | |
Gene |
RCV004590030 | SCV005080398 | likely benign | not provided | 2024-03-01 | criteria provided, single submitter | clinical testing | See Variant Classification Assertion Criteria. |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV004689962 | SCV005185772 | uncertain significance | not specified | 2024-05-15 | criteria provided, single submitter | clinical testing | Variant summary: RYR1 c.10824+8G>A alters a non-conserved nucleotide located close to a canonical splice site and therefore could affect mRNA splicing, leading to a significantly altered protein sequence. Several computational tools predict a significant impact on normal splicing: four predict the variant have no significant impact on the canonical splice-site, while three predict the variant strengthens a cryptic 5' donor site, which is located 4 nucleotides downstream from the canonical site. However, these predictions have yet to be confirmed by functional studies. The variant allele was found at a frequency of 6e-05 in 250346 control chromosomes (gnomAD). To our knowledge, no occurrence of c.10824+8G>A in individuals affected with Myopathy, RYR1-Associated and no experimental evidence demonstrating its impact on protein function have been reported. ClinVar contains an entry for this variant (Variation ID: 834318). Based on the evidence outlined above, the variant was classified as uncertain significance. |