Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV001823520 | SCV002073013 | uncertain significance | Congenital myasthenic syndrome 8 | criteria provided, single submitter | clinical testing | The missense variant p.P621L in AGRN (NM_198576.4) has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.P621L variant is observed in 1/30,518 (0.0033%) alleles from individuals of South Asian background in gnomAD Exomes and is novel (not in any individuals) in 1000 Genomes. In silico tools are contradictory in their predictions (SIFT-Tolerate, Polyphen-2-Damaging) and the residue is semiconserved across species. For these reasons, this variant has been classified as Uncertain Significance. |