Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Genomic Diagnostic Laboratory, |
RCV000202824 | SCV000258167 | uncertain significance | not specified | 2015-05-05 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001239656 | SCV001412545 | uncertain significance | Vici syndrome | 2022-08-23 | criteria provided, single submitter | clinical testing | This sequence change replaces glutamic acid, which is acidic and polar, with lysine, which is basic and polar, at codon 1358 of the EPG5 protein (p.Glu1358Lys). This variant is present in population databases (rs775481546, gnomAD 0.02%). This variant has not been reported in the literature in individuals affected with EPG5-related conditions. ClinVar contains an entry for this variant (Variation ID: 218782). 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: "Tolerated"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C0"). 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. |
Institute for Clinical Genetics, |
RCV003237764 | SCV002011464 | uncertain significance | not provided | 2021-11-03 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV004619227 | SCV005118862 | uncertain significance | Inborn genetic diseases | 2024-03-15 | criteria provided, single submitter | clinical testing | The c.4072G>A (p.E1358K) alteration is located in exon 23 (coding exon 23) of the EPG5 gene. This alteration results from a G to A substitution at nucleotide position 4072, causing the glutamic acid (E) at amino acid position 1358 to be replaced by a lysine (K). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |