Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV002227324 | SCV002506164 | uncertain significance | not provided | 2022-03-23 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV003101295 | SCV003730844 | uncertain significance | Inborn genetic diseases | 2022-12-01 | criteria provided, single submitter | clinical testing | The c.2731G>A (p.V911M) alteration is located in exon 20 (coding exon 20) of the PIEZO1 gene. This alteration results from a G to A substitution at nucleotide position 2731, causing the valine (V) at amino acid position 911 to be replaced by a methionine (M). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |
Revvity Omics, |
RCV002227324 | SCV003812905 | uncertain significance | not provided | 2022-11-23 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002227324 | SCV004561916 | benign | not provided | 2023-05-29 | criteria provided, single submitter | clinical testing | |
Clinical Genomics Laboratory, |
RCV005051951 | SCV005685481 | uncertain significance | Lymphatic malformation 6 | 2024-10-01 | criteria provided, single submitter | clinical testing | A PIEZO1 c.2731G>A (p.Val911Met) variant was identified at a near heterozygous allelic fraction of 48.85%, a frequency that may be consistent with germline origin. This variant, to our knowledge, has not been reported in the medical literature and is only observed on 60/1,549,666 alleles in the general population (gnomAD v.4.1.0), indicating it is not a common variant. This variant has been reported in the ClinVar database as a benign variant by one submitter and a variant of uncertain significance by three submitters, all in a germline state (Clinvar ID: 1679445). Computational predictors suggest that the variant does not impact PIEZO1 function. Due to limited information, and based on ACMG/AMP guidelines for variant interpretation (Richards S et al., PMID: 25741868), the clinical significance of the PIEZO1 c.2731G>A (p.Val911Met) variant is uncertain at this time. |