Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002790725 | SCV003033781 | pathogenic | Hereditary spastic paraplegia 63; Pontocerebellar hypoplasia type 9 | 2022-03-19 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Ala47Glyfs*147) in the AMPD2 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in AMPD2 are known to be pathogenic (PMID: 23911318). For these reasons, this variant has been classified as Pathogenic. Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. This variant has not been reported in the literature in individuals affected with AMPD2-related conditions. This variant is not present in population databases (gnomAD no frequency). |