Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003107859 | SCV003783235 | pathogenic | not provided | 2022-07-26 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with glycine, which is neutral and non-polar, at codon 18 of the H3F3A protein (p.Arg18Gly). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with Bryant-Li-Bhoj neurodevelopmental syndrome (PMID: 33268356). In at least one individual the variant was observed to be de novo. This variant is also known as p.R17G. ClinVar contains an entry for this variant (Variation ID: 1339283). Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Benign"; Align-GVGD: "Class C65"). For these reasons, this variant has been classified as Pathogenic. |
OMIM | RCV001823808 | SCV002073383 | pathogenic | Bryant-Li-Bhoj neurodevelopmental syndrome 1 | 2022-02-01 | no assertion criteria provided | literature only |