Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Baylor Genetics | RCV000007030 | SCV005058482 | pathogenic | Congenital myasthenic syndrome 5 | 2024-03-27 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000007030 | SCV005777314 | pathogenic | Congenital myasthenic syndrome 5 | 2024-04-08 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg282*) in the COLQ gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in COLQ are known to be pathogenic (PMID: 22678886). This variant is present in population databases (rs121908922, gnomAD 0.0009%). This premature translational stop signal has been observed in individual(s) with endplate acetylcholinesterase deficiency (PMID: 9689136). ClinVar contains an entry for this variant (Variation ID: 6651). For these reasons, this variant has been classified as Pathogenic. |
OMIM | RCV000007030 | SCV000027226 | pathogenic | Congenital myasthenic syndrome 5 | 1998-08-04 | no assertion criteria provided | literature only |