Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Fulgent Genetics, |
RCV002504810 | SCV002794558 | pathogenic | Familial visceral amyloidosis, Ostertag type; Hypoalphalipoproteinemia, primary, 2; Hypoalphalipoproteinemia, primary, 2, intermediate | 2021-10-13 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV003556050 | SCV004294953 | pathogenic | not provided | 2023-03-24 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Experimental studies have shown that this missense change affects APOA1 function (PMID: 17665932, 21296086, 22952757, 23233678, 27464946). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt APOA1 protein function. ClinVar contains an entry for this variant (Variation ID: 17917). This variant is also known as glycine to arginine-26 substitution, ApoA-I Iowa, or G26R. This missense change has been observed in individual(s) with autosomal dominant systemic amyloidosis (PMID: 2123470). It has also been observed to segregate with disease in related individuals. This variant is not present in population databases (gnomAD no frequency). This sequence change replaces glycine, which is neutral and non-polar, with arginine, which is basic and polar, at codon 50 of the APOA1 protein (p.Gly50Arg). |
OMIM | RCV000019506 | SCV000039803 | pathogenic | Familial amyloid polyneuropathy, Iowa type | 1990-10-01 | no assertion criteria provided | literature only |