Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001837950 | SCV002098218 | likely pathogenic | not provided | 2022-04-19 | criteria provided, single submitter | clinical testing | Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Has not been previously published as pathogenic or benign to our knowledge; This variant is associated with the following publications: (PMID: 24785942) |
Molecular Diagnostics Lab, |
RCV000552933 | SCV005187389 | likely pathogenic | Hypomyelinating leukodystrophy 6 | 2020-12-28 | criteria provided, single submitter | clinical testing | This missense variant (c.1062C>G, P.Cys354Trp) was not observed in population databases (gnomAD). It has been reported in the literature (PMID 24785942) and variant prediction programs suggest a deleterious effect, although no functional studies have been published. It was found in an affected individual and his asymptomatic father, who is believed to be mosaic for the change. |
Undiagnosed Diseases Network, |
RCV000552933 | SCV000622155 | likely pathogenic | Hypomyelinating leukodystrophy 6 | 2016-04-18 | no assertion criteria provided | clinical testing | Patient's MRI consistent with the findings in H-ABC. Previous MRIs did not show these findings, as this is a progressive disorder. Likely pathogenicity is based on laboratory report as well as the clinical findings correlating with the diagnosis. |