Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV000756188 | SCV000883917 | uncertain significance | not provided | 2024-01-26 | criteria provided, single submitter | clinical testing | The FLNB c.6515G>A; p.Arg2172His variant (rs139846706), to our knowledge, is not reported in the medical literature but is reported in ClinVar (Variation ID: 618136). This variant is observed in the general population with an overall allele frequency of 0.01% (29/282724 alleles) in the Genome Aggregation Database (v2.1.1). Computational analyses are uncertain whether this variant is neutral or deleterious (REVEL: 0.158). Due to the occurrence of p.Arg2172His in the general adult population, this variant is unlikely to be associated with a severe autosomal dominant FLNB-related disease such as atelosteogenesis types I (AOI) and III (AOIII) or Piepkorn osteochondrodysplasia (Robertson 2020); however, due to limited information regarding the p.Arg2172His variant, its clinical significance for a milder or autosomal recessive condition cannot be excluded. REFERENCES Robertson S. FLNB Disorders. 2008 Oct 9 (Updated 2020 Feb 13). In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews (Internet). Seattle (WA): University of Washington, Seattle; 1993-2020. Available from: https://www.ncbi.nlm.nih.gov/books/NBK2534/ |
Ce |
RCV000756188 | SCV001153988 | uncertain significance | not provided | 2016-05-01 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000756188 | SCV002235165 | likely benign | not provided | 2024-07-31 | criteria provided, single submitter | clinical testing | |
Genome Diagnostics Laboratory, |
RCV002279511 | SCV002566699 | uncertain significance | Connective tissue disorder | 2021-10-28 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002533120 | SCV003742464 | likely benign | Inborn genetic diseases | 2022-11-30 | criteria provided, single submitter | clinical testing | This alteration is classified as likely benign based on a combination of the following: seen in unaffected individuals, population frequency, intact protein function, lack of segregation with disease, co-occurrence, RNA analysis, in silico models, amino acid conservation, lack of disease association in case-control studies, and/or the mechanism of disease or impacted region is inconsistent with a known cause of pathogenicity. |
Gene |
RCV000756188 | SCV003798929 | uncertain significance | not provided | 2023-02-02 | criteria provided, single submitter | clinical testing | In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Has not been previously published as pathogenic or benign to our knowledge |