Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
MGZ Medical Genetics Center | RCV002290332 | SCV002580376 | uncertain significance | X-linked myopathy with postural muscle atrophy | 2021-10-04 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002290332 | SCV004268116 | uncertain significance | X-linked myopathy with postural muscle atrophy | 2023-07-08 | criteria provided, single submitter | clinical testing | This variant is not present in population databases (gnomAD no frequency). This sequence change replaces threonine, which is neutral and polar, with asparagine, which is neutral and polar, at codon 190 of the FHL1 protein (p.Thr190Asn). This variant has not been reported in the literature in individuals affected with FHL1-related conditions. ClinVar contains an entry for this variant (Variation ID: 1709990). An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be tolerated. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |