Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002233696 | SCV000835770 | pathogenic | Familial hypercholesterolemia | 2020-09-17 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Loss-of-function variants in LDLR are known to be pathogenic (PMID: 20809525). This variant has been reported in an individual affected with familial hypercholesterolemia (PMID: 23430853). This variant is also known as  c.1849delA (p.Val597Tyrfs45X) in the literature. This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Val618Tyrfs*47) in the LDLR gene. It is expected to result in an absent or disrupted protein product. |
Quest Diagnostics Nichols Institute San Juan Capistrano | RCV001800859 | SCV002046536 | pathogenic | not provided | 2020-12-30 | criteria provided, single submitter | clinical testing | This frameshift variant causes the premature termination of LDLR protein synthesis. It has been reported in a child affected with homozygous familial hypercholesteremia in the published literature (PMID: 23430853 (2011)). Therefore, the variant is classified as pathogenic. |