Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000725864 | SCV000340079 | pathogenic | not provided | 2016-03-03 | criteria provided, single submitter | clinical testing | |
Counsyl | RCV000310267 | SCV000485600 | likely pathogenic | Autosomal recessive limb-girdle muscular dystrophy type 2A | 2016-10-28 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000310267 | SCV001405152 | pathogenic | Autosomal recessive limb-girdle muscular dystrophy type 2A | 2023-05-11 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 286591). This premature translational stop signal has been observed in individuals with autosomal recessive limb-girdle muscular dystrophy (PMID: 9150160, 21204801). This variant is present in population databases (rs746935735, gnomAD 0.0009%). This sequence change creates a premature translational stop signal (p.Ile135Leufs*4) in the CAPN3 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in CAPN3 are known to be pathogenic (PMID: 10330340, 15689361). |