Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV000495878 | SCV002817121 | uncertain significance | Hypercholesterolemia, familial, 1 | 2022-08-29 | reviewed by expert panel | curation | The NM_000527.4(LDLR):c.-97G>A is a LDLR-AS1 Non Coding Transcript Variant and is classified as Uncertain significance - insufficient evidence for Familial Hypercholesterolemia by applying evidence codes PM2 and BP4 as defined by the ClinGen Familial Hypercholesterolemia Expert Panel LDLR-specific variant curation guidelines (https://doi.org/10.1016/j.gim.2021.09.012). The supporting evidence is as follow: PM2_Met : absent from controls in GnomAD (gnomAD v2.1.1). PP4_Met : 1 index case with Dutch lipid clinic network >=6 PS4_supporting: variant met PM2 and was found in 1 index case with Dutch lipid clinic network >=6 (sequenced by progenica) from M.Arca Lab and 1 patient from Lille University & CHRU Lille (ClinVar entry) with Dutch Lipid Clinic Scoring of Probable FH |
U4M - |
RCV000495878 | SCV000583620 | likely pathogenic | Hypercholesterolemia, familial, 1 | 2017-03-30 | criteria provided, single submitter | clinical testing | ACMG Guidelines: Likely Pathogenic (ii) |
Color Diagnostics, |
RCV000776578 | SCV000912191 | likely benign | Familial hypercholesterolemia | 2019-08-11 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000776578 | SCV003451328 | uncertain significance | Familial hypercholesterolemia | 2024-12-12 | criteria provided, single submitter | clinical testing | This variant occurs in a non-coding region of the LDLR gene. It does not change the encoded amino acid sequence of the LDLR protein. This variant is not present in population databases (gnomAD no frequency). This variant has been observed in individual(s) with familial hypercholesterolemia (PMID: 28965616). ClinVar contains an entry for this variant (Variation ID: 430743). Experimental studies and prediction algorithms are not available or were not evaluated, and the functional significance of this variant is currently unknown. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
All of Us Research Program, |
RCV000495878 | SCV004829717 | likely benign | Hypercholesterolemia, familial, 1 | 2024-02-22 | criteria provided, single submitter | clinical testing |