Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000396191 | SCV000338661 | uncertain significance | not provided | 2016-01-23 | criteria provided, single submitter | clinical testing | |
Medical Molecular Genetics Department, |
RCV004764924 | SCV005374907 | likely pathogenic | Autosomal recessive limb-girdle muscular dystrophy type 2A | 2024-10-10 | criteria provided, single submitter | clinical testing | By applying ACMG guidelines: According to insilico studies, the variant is classified as deleterious (PP3),our study patient’s clinical phenotype is typically correlated to the disease (PP4), it showed an extremely low frequency in gnomAD population databases (PS4) additionally, by segregation analysis: the affected proband showed the same variant at the heterozygous status coupled with the previously reported variant in CAPN3 (NM_000070.2:c.1343G>A p.Arg448His) at the heterozygous form, while her healthy sibling showed the wild genotype for both alleles and parents were carriers (showed one allelic heterozygous status) (PP1). according to this data it is considered as likely pathogenic by ACMG guidlines. |