Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000824136 | SCV000965021 | uncertain significance | Hereditary spastic paraplegia 63; Pontocerebellar hypoplasia type 9 | 2022-03-23 | criteria provided, single submitter | clinical testing | This sequence change replaces isoleucine, which is neutral and non-polar, with threonine, which is neutral and polar, at codon 497 of the AMPD2 protein (p.Ile497Thr). This variant is present in population databases (rs149171366, gnomAD 0.007%). This variant has not been reported in the literature in individuals affected with AMPD2-related conditions. ClinVar contains an entry for this variant (Variation ID: 665782). 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: "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. |
Ambry Genetics | RCV002536013 | SCV003710188 | uncertain significance | Inborn genetic diseases | 2022-10-03 | criteria provided, single submitter | clinical testing | The c.1490T>C (p.I497T) alteration is located in exon 11 (coding exon 11) of the AMPD2 gene. This alteration results from a T to C substitution at nucleotide position 1490, causing the isoleucine (I) at amino acid position 497 to be replaced by a threonine (T). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |
Genome- |
RCV003344080 | SCV004050583 | uncertain significance | Hereditary spastic paraplegia 63 | 2023-04-11 | criteria provided, single submitter | clinical testing | |
Genome- |
RCV003344081 | SCV004050584 | uncertain significance | Pontocerebellar hypoplasia type 9 | 2023-04-11 | criteria provided, single submitter | clinical testing |