Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000728541 | SCV000856132 | pathogenic | not provided | 2017-08-03 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001210761 | SCV001382264 | pathogenic | Alagille syndrome due to a JAG1 point mutation | 2022-02-09 | criteria provided, single submitter | clinical testing | This sequence change affects the initiator methionine of the JAG1 mRNA. The next in-frame methionine is located at codon 42. For these reasons, this variant has been classified as Pathogenic. This variant disrupts a region of the JAG1 protein in which other variant(s) (p.Leu37Ser) have been determined to be pathogenic (PMID: 11157803, 11180599). This suggests that this is a clinically significant region of the protein, and that variants that disrupt it are likely to be disease-causing. ClinVar contains an entry for this variant (Variation ID: 213528). This variant has not been reported in the literature in individuals affected with JAG1-related conditions. This variant is not present in population databases (gnomAD no frequency). |