Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Molecular Diagnostics Lab, |
RCV000011845 | SCV005088464 | likely pathogenic | Pelizaeus-Merzbacher disease | 2021-12-09 | criteria provided, single submitter | clinical testing | This intronic variant (c.453+4A>G) has not been observed in population databases (gnomAD). It has been described in the literature, and functional studies indicate a deleterious effect on expression of the PLP protein (PMID 16287154, PMID 11071483). |
OMIM | RCV000011845 | SCV000032078 | pathogenic | Pelizaeus-Merzbacher disease | 2000-10-24 | no assertion criteria provided | literature only |