Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
CGC Genetics, |
RCV004549039 | SCV005043273 | likely pathogenic | Syndromic intellectual disability | 2024-04-30 | no assertion criteria provided | clinical testing | The variant NM_133330.3:c.1589del p.(Lys530Argfs*68), detected in heterozygosity in the NSD2 gene, has not been described in the literature nor in gnomAD at the time of this submission. It is a frameshift variant, located in exon 9 (of 24), which introduces a premature stop codon, which is predicted to lead to the creation of a truncated protein and/or a reduction in its expression due to mRNA degradation. In addition, another frameshift variant with a nearby premature stop codon is reported in ClinVar as pathogenic (ClinVar ID: 1333176). With the information currently available and current recommendations, this variant should be classified as a probably pathogenic variant. ACMG codes: PVS1; PM2_supp. |