Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV003331538 | SCV004048272 | likely pathogenic | Developmental and epileptic encephalopathy, 76 | criteria provided, single submitter | clinical testing | The frameshift variant c.695dup (p.Asn233LysfsTer17) in ACTL6B gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Asn233LysfsTer17 variant is novel (not in any individuals) in 1000 Genomes and allele frequency of 0.008648% is reported in gnomAD. This variant causes a frameshift starting with codon Asparagine 233, changes this amino acid to Lysine residue, and creates a premature Stop codon at position 17 of the new reading frame, denoted p.Asn233LysfsTer17. For these reasons, this variant has been classified as Likely Pathogenic . | |
Houlden Lab, |
RCV003331538 | SCV003920666 | pathogenic | Developmental and epileptic encephalopathy, 76 | no assertion criteria provided | research |