Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Molecular Genetics Department, |
RCV003985962 | SCV004801775 | likely pathogenic | Rubinstein-Taybi syndrome due to CREBBP mutations | criteria provided, single submitter | clinical testing | A previously undescribed nucleotide variant creates a frameshift p.Arg1091ThrfsTer37 in the CREBBP gene. The variant was observed in heterozygous state in an individual affected with agenesia of corpus callosum, megacytis, neonatal seizures, and dysmorphic features. Loss-of-function variants are reported in patients with Rubinstein-Taybi syndrome 1, 180849. The variant is not present in population database (gnomAD no frequency). In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as likely pathogenic. |