Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001341985 | SCV001535884 | uncertain significance | Hypertrophic cardiomyopathy 14 | 2020-10-21 | criteria provided, single submitter | clinical testing | 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. This variant has been observed in individual(s) with hypoplastic left heart syndrome (PMID: 27789736). This variant is present in population databases (rs372270600, ExAC 0.01%). This sequence change creates a premature translational stop signal (p.Glu1754*) in the MYH6 gene. It is expected to result in an absent or disrupted protein product. However, the current clinical and genetic evidence is not sufficient to establish whether loss-of-function variants in MYH6 cause disease. |
Prevention |
RCV004734128 | SCV005363932 | uncertain significance | MYH6-related disorder | 2024-09-11 | no assertion criteria provided | clinical testing | The MYH6 c.5260G>T variant is predicted to result in premature protein termination (p.Glu1754*). This variant was reported in an individual with hypoplastic left heart syndrome (Tomita-Mitchell et al 2016. PubMed ID: 27789736). This variant is reported in 0.012% of alleles in individuals of African descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |