Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Center for Genomics, |
RCV000768016 | SCV000898808 | uncertain significance | Chédiak-Higashi syndrome | 2021-03-30 | criteria provided, single submitter | clinical testing | LYST NM_000081.3 exon 22 p.Gly2062Glu (c.6185G>A): This variant has not been reported in the literature but is present in 2/111072 European alleles in the Genome Aggregation Database (http://gnomad.broadinstitute.org/rs756651685). Evolutionary conservation and computational predictive tools for this variant are unclear. In summary, data on this variant is insufficient for disease classification. Therefore, the clinical significance of this variant is uncertain. |
Labcorp Genetics |
RCV000768016 | SCV001533244 | uncertain significance | Chédiak-Higashi syndrome | 2021-08-28 | criteria provided, single submitter | clinical testing | This sequence change replaces glycine with glutamic acid at codon 2062 of the LYST protein (p.Gly2062Glu). The glycine residue is moderately conserved and there is a moderate physicochemical difference between glycine and glutamic acid. This variant is present in population databases (rs756651685, ExAC 0.003%). This variant has not been reported in the literature in individuals affected with LYST-related conditions. ClinVar contains an entry for this variant (Variation ID: 625976). Algorithms developed to predict the effect of missense changes on protein structure and function (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be tolerated. 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. |
Mayo Clinic Laboratories, |
RCV002261200 | SCV002541721 | uncertain significance | not provided | 2021-04-05 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002533932 | SCV003533890 | uncertain significance | Inborn genetic diseases | 2022-11-21 | criteria provided, single submitter | clinical testing | The c.6185G>A (p.G2062E) alteration is located in exon 22 (coding exon 20) of the LYST gene. This alteration results from a G to A substitution at nucleotide position 6185, causing the glycine (G) at amino acid position 2062 to be replaced by a glutamic acid (E). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |