Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Molecular Genetics and NGS Laboratory, |
RCV004698762 | SCV005200476 | likely pathogenic | Mandibulofacial dysostosis-microcephaly syndrome | 2024-08-14 | criteria provided, single submitter | clinical testing | Null variant (nonsense) in gene EFTUD2, predicted to cause NMD. Loss-of-function is a known mechanism of disease. The exon affects 1 functional domain: UniProt protein U5S1_HUMAN domain 'tr-type G'. The truncated region contains 132 pathogenic variants (PVS1). The variant has not been found in genomAD exomes or in genomAD genomes. We identified this variant in heterozygosity in a 30-year-old man with a phenotype of mandibulofacial dysostosis. |