Total submissions: 18
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001082494 | SCV000071825 | benign | Hereditary breast ovarian cancer syndrome | 2024-02-01 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV000131573 | SCV000186579 | benign | Hereditary cancer-predisposing syndrome | 2014-11-19 | criteria provided, single submitter | clinical testing | This alteration is classified as 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 |
RCV000679155 | SCV000210560 | likely benign | not provided | 2021-11-24 | criteria provided, single submitter | clinical testing | This variant is associated with the following publications: (PMID: 22476429, 25248401, 20104584, 18559594, 21990134, 21952622, 27208206, 24763404, 20167696) |
Counsyl | RCV000031322 | SCV000220591 | likely benign | Breast-ovarian cancer, familial, susceptibility to, 2 | 2014-08-12 | criteria provided, single submitter | literature only | |
ARUP Laboratories, |
RCV000679155 | SCV000602832 | likely benign | not provided | 2019-11-16 | criteria provided, single submitter | clinical testing | |
Institute for Biomarker Research, |
RCV000131573 | SCV000679708 | likely benign | Hereditary cancer-predisposing syndrome | 2017-07-12 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV000679155 | SCV000805653 | likely benign | not provided | 2017-08-17 | criteria provided, single submitter | clinical testing | |
Color Diagnostics, |
RCV000131573 | SCV000910624 | benign | Hereditary cancer-predisposing syndrome | 2015-12-15 | criteria provided, single submitter | clinical testing | |
Illumina Laboratory Services, |
RCV001109419 | SCV001266758 | uncertain significance | Fanconi anemia complementation group D1 | 2018-08-30 | criteria provided, single submitter | clinical testing | This variant was observed as part of a predisposition screen in an ostensibly healthy population. A literature search was performed for the gene, cDNA change, and amino acid change (where applicable). No publications were found based on this search. Allele frequency data from public databases did not allow this variant to be ruled in or out of causing disease. Therefore, this variant is classified as a variant of unknown significance. |
Illumina Laboratory Services, |
RCV000031322 | SCV001273003 | uncertain significance | Breast-ovarian cancer, familial, susceptibility to, 2 | 2018-08-30 | criteria provided, single submitter | clinical testing | This variant was observed as part of a predisposition screen in an ostensibly healthy population. A literature search was performed for the gene, cDNA change, and amino acid change (where applicable). No publications were found based on this search. Allele frequency data from public databases did not allow this variant to be ruled in or out of causing disease. Therefore, this variant is classified as a variant of unknown significance. |
Genetic Services Laboratory, |
RCV001818198 | SCV002068640 | likely benign | not specified | 2019-03-18 | criteria provided, single submitter | clinical testing | |
Sema4, |
RCV000131573 | SCV002533233 | likely benign | Hereditary cancer-predisposing syndrome | 2021-07-22 | criteria provided, single submitter | curation | |
Center for Genomic Medicine, |
RCV001818198 | SCV002550279 | likely benign | not specified | 2023-08-15 | criteria provided, single submitter | clinical testing | |
University of Washington Department of Laboratory Medicine, |
RCV000131573 | SCV003851406 | likely benign | Hereditary cancer-predisposing syndrome | 2023-03-23 | criteria provided, single submitter | curation | Missense variant in a coldspot region where missense variants are very unlikely to be pathogenic (PMID:31911673). |
Sharing Clinical Reports Project |
RCV000031322 | SCV000053927 | benign | Breast-ovarian cancer, familial, susceptibility to, 2 | 2011-03-03 | no assertion criteria provided | clinical testing | |
Breast Cancer Information Core |
RCV000031322 | SCV000145875 | uncertain significance | Breast-ovarian cancer, familial, susceptibility to, 2 | 2002-05-29 | no assertion criteria provided | clinical testing | |
Department of Pathology and Laboratory Medicine, |
RCV000031322 | SCV000591745 | likely benign | Breast-ovarian cancer, familial, susceptibility to, 2 | no assertion criteria provided | clinical testing | The p.Ala487Glu variant was identified in 4 of 5096 proband chromosomes (frequency: 0.001) from individuals with breast or ovarian cancer (Borg 2010, Lindor 2012, Soegaard 2008); however, control chromosomes were not evaluated in these studies, thus the prevalence of this variant in the general population could not be determined. The variant was also identified in dbSNP (ID: rs56390402), the ClinVar database (classified as a benign variant by Ambry Genetics and the Sharing Clinical Reports Project (derived from Myriad reports); classified as likely benign by GeneDx), the BIC database (21X with unknown clinical importance), and UMD (6X as an unclassified variant). In UMD the variant was identified in one sample with a co-occurring pathogenic BRCA2 variant (c.6816_6820delAAGAG (p.Gly2274AlafsX17)), and in another sample with a co-occurring pathogenic BRCA1 variant (c.4327C>T (p.Arg1443X)), increasing the likelihood that the p.Ala487Glu variant does not have clinical significance. The variant was listed in the Exome Aggregation Consortium (ExAC) database (released Oct 20th, 2014) in 6 of 66248 chromosomes (frequency: 0.00009) from a population of European (Non-Finnish) individuals, and was also found in 1 of 8598 European American chromosomes in the Exome Variant Server ESP project. This low number of observations and low frequency is not substantive enough to determine the prevalence of the variant in the general population and its relationship to disease. The variant occurs outside of the splicing consensus sequence and one of five in silico or computational prediction software programs (SpliceSiteFinder, MaxEntScan, NNSPLICE, GeneSplicer, HumanSpliceFinder) predicts a difference in splicing, but this information is not very predictive of pathogenicity. The p.Ala487 residue is not conserved in mammals and other organisms, and computational analyses (PolyPhen-2, SIFT, AlignGVGD, BLOSUM, MutationTaster) provide inconsistent predictions regarding the impact to the protein; this information is not very predictive of pathogenicity. In summary, based on the above information, the clinical significance of this variant cannot be determined with certainty at this time although we would lean towards a more benign role for this variant. This variant is classified as predicted benign. | |
BRCAlab, |
RCV000031322 | SCV004244218 | likely benign | Breast-ovarian cancer, familial, susceptibility to, 2 | 2020-03-02 | no assertion criteria provided | clinical testing |