Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV000566636 | SCV000672469 | uncertain significance | Hereditary cancer-predisposing syndrome | 2023-01-10 | criteria provided, single submitter | clinical testing | The p.R1113Q variant (also known as c.3338G>A), located in coding exon 20 of the ALK gene, results from a G to A substitution at nucleotide position 3338. The arginine at codon 1113 is replaced by glutamine, an amino acid with highly similar properties. This amino acid position is highly conserved in available vertebrate species. In addition, this alteration is predicted to be deleterious by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Labcorp Genetics |
RCV000647454 | SCV000769250 | likely benign | Neuroblastoma, susceptibility to, 3 | 2024-01-28 | criteria provided, single submitter | clinical testing | |
Sema4, |
RCV000566636 | SCV002528474 | likely benign | Hereditary cancer-predisposing syndrome | 2021-05-06 | criteria provided, single submitter | curation | |
Baylor Genetics | RCV000647454 | SCV004198601 | uncertain significance | Neuroblastoma, susceptibility to, 3 | 2024-03-29 | criteria provided, single submitter | clinical testing | |
Gene |
RCV001354522 | SCV005325476 | uncertain significance | not provided | 2024-02-21 | 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 |
Department of Pathology and Laboratory Medicine, |
RCV001354522 | SCV001549163 | uncertain significance | not provided | no assertion criteria provided | clinical testing | The ALK p.R1113Q variant was not identified in the literature but was identified in dbSNP (ID: rs199987354) and ClinVar (classified as uncertain significance by Ambry Genetics and as likely benign by Invitae). The variant was identified in control databases in 14 of 282560 chromosomes at a frequency of 0.00004955, and was observed at the highest frequency in the Ashkenazi Jewish population in 11 of 10368 chromosomes (freq: 0.001061) (Genome Aggregation Database March 6, 2019, v2.1.1). The p.R1113 residue is conserved in mammals and more distantly related organisms, and computational analyses (MUT Assesor, PolyPhen-2, SIFT, MutationTaster, Revel, FATHMM, MetaLR, DANN) suggest that the variant may impact the protein; however, this information is not predictive enough to assume pathogenicity. The variant occurs outside of the splicing consensus sequence and in silico or computational prediction software programs (Splice AI exome) do not predict a difference in splicing. In summary, based on the above information the clinical significance of this variant cannot be determined with certainty at this time. This variant is classified as a variant of uncertain significance. |