Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV000508827 | SCV005375283 | uncertain significance | Hypercholesterolemia, familial, 1 | 2023-03-27 | reviewed by expert panel | curation | The NM_000527.5(LDLR):c.188G>T (p.Cys63Phe) variant is classified as Uncertain significance - insufficient evidence for Familial Hypercholesterolemia by applying ACMG/AMP evidence codes PM1, PM2 and PP3 as defined by the ClinGen Familial Hypercholesterolemia Expert Panel LDLR-specific variant curation guidelines (specification version 1.2) on 27 March 2023. The supporting evidence is as follows: PM2: This variant is absent from gnomAD (gnomAD v2.1.1). PP3: REVEL = 0.921. PM1: Variant is missense, meets PM2, and alters Cys63, one of the 60 highly conserved cysteine residues. |
Laboratorium voor Moleculaire Diagnostiek Experimentele Vasculaire Geneeskunde, |
RCV000508827 | SCV000606035 | pathogenic | Hypercholesterolemia, familial, 1 | no assertion criteria provided | research |