Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000796057 | SCV000935548 | likely benign | Ataxia-telangiectasia syndrome | 2023-01-19 | criteria provided, single submitter | clinical testing | |
Color Diagnostics, |
RCV001188744 | SCV001355880 | uncertain significance | Hereditary cancer-predisposing syndrome | 2023-12-04 | criteria provided, single submitter | clinical testing | This missense variant replaces isoleucine with phenylalanine at codon 1022 of the ATM protein. Computational prediction suggests that this variant may not impact protein structure and function (internally defined REVEL score threshold <= 0.5, PMID: 27666373). To our knowledge, functional studies have not been reported for this variant. This variant has not been reported in individuals affected with ATM-related disorders in the literature. This variant has not been identified in the general population by the Genome Aggregation Database (gnomAD). The available evidence is insufficient to determine the role of this variant in disease conclusively. Therefore, this variant is classified as a Variant of Uncertain Significance. |
Ambry Genetics | RCV001188744 | SCV005037732 | uncertain significance | Hereditary cancer-predisposing syndrome | 2023-11-16 | criteria provided, single submitter | clinical testing | The p.I1022F variant (also known as c.3064A>T), located in coding exon 19 of the ATM gene, results from an A to T substitution at nucleotide position 3064. The isoleucine at codon 1022 is replaced by phenylalanine, an amino acid with highly similar properties. This amino acid position is not well conserved in available vertebrate species. In addition, this alteration is predicted to be tolerated by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Natera, |
RCV000796057 | SCV002076625 | uncertain significance | Ataxia-telangiectasia syndrome | 2020-12-17 | no assertion criteria provided | clinical testing |