Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ce |
RCV002293068 | SCV002585932 | likely pathogenic | not provided | 2022-07-01 | criteria provided, single submitter | clinical testing | PLA2G6: PM2, PM3:Supporting, PP1, PP3, PS3:Supporting |
Illumina Laboratory Services, |
RCV003408219 | SCV004123227 | uncertain significance | PLA2G6-associated neurodegeneration | 2023-08-03 | criteria provided, single submitter | clinical testing | The PLA2G6 c.2035-926G>A variant occurs in an intron. This variant has been reported in the literature in trans with a missense variant in an individual with progressive neurologic disease (PMID: 35247231). The c.2035-926G>A variant is not observed in version 2.1.1 or version 3.1.2 of the Genome Aggregation Database. A functional study conducted in patient fibroblast cells demonstrated that the c.2035-926G>A variant results in abnormal splicing (PMID: 35247231). This variant is observed in a homozygous state. Based on the available evidence, the c.2035-926G>A variant is classified as a variant of uncertain significance for PLA2G6-associated neurodegeneration. |
Undiagnosed Diseases Network, |
RCV003164492 | SCV003915649 | uncertain significance | Infantile neuroaxonal dystrophy | 2022-04-02 | no assertion criteria provided | clinical testing |