Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000702844 | SCV000831715 | likely benign | RASopathy | 2022-12-06 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV000779849 | SCV000916702 | benign | not specified | 2018-09-02 | criteria provided, single submitter | clinical testing | Variant summary: BRAF c.68T>A (p.Met23Lys) results in a non-conservative amino acid change in the encoded protein sequence. Three of four in-silico tools predict a benign effect of the variant on protein function (Mutation Taster not included due to low p-value). The variant allele was found at a frequency of 8.3e-05 in 121194 control chromosomes, predominantly at a frequency of 0.00045 within the South Asian subpopulation in the gnomAD database. The observed variant frequency within South Asian control individuals in the gnomAD database is approximately 180-folds higher than the estimated maximal expected allele frequency for a pathogenic variant in BRAF causing Noonan Syndrome and Related Conditions phenotype (2.5e-06), strongly suggesting that the variant is a benign polymorphism found primarily in populations of South Asian origin. To our knowledge, no occurrence of c.68T>A in individuals affected with Noonan Syndrome and Related Conditions and no experimental evidence demonstrating its impact on protein function have been reported. No clinical diagnostic laboratories have submitted clinical-significance assessments for this variant to ClinVar after 2014. Based on the evidence outlined above, the variant was classified as benign. |
Prevention |
RCV004752999 | SCV005367243 | uncertain significance | BRAF-related disorder | 2024-08-28 | no assertion criteria provided | clinical testing | The BRAF c.68T>A variant is predicted to result in the amino acid substitution p.Met23Lys. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.045% of alleles in individuals of South Asian descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |