Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Pittsburgh Clinical Genomics Laboratory, |
RCV004785004 | SCV005397383 | pathogenic | Cardiac malformation, cleft lip/palate, microcephaly, and digital anomalies | 2021-06-11 | criteria provided, single submitter | clinical testing | This sequence variant is a single nucleotide substitution (C>T) that results in the generation of an early termition codon at position 152 of the MEIS2 protein. This variant is predicted to generate a non-functiol allele through the expression of a truncated protein or a loss of MEIS2 expression due to nonsense mediated decay. This is a de novo variant that has not been previously reported in individuals with MEIS2-related disease, in control population datasets (gnomAD database 0/~250,000 alleles), or in clinical variant repositories (ClinVar), to our knowledge. Because truncating variants are a known mechanism of disease for MEIS2 (PMID: 27225850), we consider this variant to be pathogenic. ACMG Criteria: PM2, PS2, PVS1 |