Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Molecular Diagnostics Lab, |
RCV004595779 | SCV005088471 | likely pathogenic | Pelizaeus-Merzbacher disease | 2022-01-24 | criteria provided, single submitter | clinical testing | This intronic variant (c.454-314T>A) has not been observed in population databases (gnomAD). It has been described in the literature (PMID 24890387, PMID 26125040), and functional studies have shown a deleterious effect on protein expression. It has been found in six affected males over two generations in a single family. |