Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000971012 | SCV001118626 | benign | not provided | 2023-10-08 | criteria provided, single submitter | clinical testing | |
Mendelics | RCV000013468 | SCV001138015 | benign | Corneal dystrophy, Fuchs endothelial, 6 | 2023-12-24 | criteria provided, single submitter | clinical testing | |
H3Africa Consortium | RCV001777136 | SCV002014660 | benign | not specified | 2020-10-28 | criteria provided, single submitter | research | While the frequency of the alternate allele in gnoMAD v2.0.2 is 0.083, its frequency in African populations is >5%. This suggests that previous classifications of this variant as pathogenic are in error. |
OMIM | RCV000013468 | SCV000033715 | pathogenic | Corneal dystrophy, Fuchs endothelial, 6 | 2010-01-01 | no assertion criteria provided | literature only | |
Reproductive Health Research and Development, |
RCV000013468 | SCV001142408 | benign | Corneal dystrophy, Fuchs endothelial, 6 | 2020-01-06 | no assertion criteria provided | curation | The ZEB1 gene is known as TCF8 in the published literature (PMID: 20036349). TCF8NM_030751.5:c.233A>C in the ZEB1 gene has an allele frequency of 0.052 in African subpopulation in the gnomAD database. 30 homozygous occurrences are observed in the gnomAD database. Since the Fuchs Corneal Dystrophy was reported as Late-Onset, we determined to not apply the number of homozygousity as a strong benign evidence. In addition, Riazuddin et al. reported a patient with Late-Onset Fuchs Corneal Dystrophy barboring p.N78T (PMID: 20036349). These evidence suggest the variant to be classified as benign. ACMG/AMP criteria applied: BA1. |
Prevention |
RCV003982838 | SCV004796454 | likely benign | ZEB1-related disorder | 2022-05-25 | no assertion criteria provided | clinical testing | This variant is classified as likely benign based on ACMG/AMP sequence variant interpretation guidelines (Richards et al. 2015 PMID: 25741868, with internal and published modifications). |