Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV000032863 | SCV001335410 | pathogenic | Intellectual disability, autosomal dominant 6 | 2017-04-04 | criteria provided, single submitter | clinical testing | This variant was identified as de novo (maternity and paternity confirmed). |
Breda Genetics srl | RCV000032863 | SCV001443275 | pathogenic | Intellectual disability, autosomal dominant 6 | 2020-09-01 | criteria provided, single submitter | clinical testing | The variant c.2172-2A>G in the GRIN2B gene is reported as pathogenic for autosomal dominant mental retardation 6 in ClinVar (Variation ID: 39659). The variant affects the acceptor splice site of intron 10 and is therefore highly likely to impact the splicing process by causing the exclusion of the following exons from the mature transcript and the translation of an aberrant protein or a shift in the reading frame. There is no information on frequency in gnomAD, 1000 Genomes or NHLI Exome Sequencing Project (ESP). The c.2172-2A>G de novo pathogenic variant has already been reported by O’Roak et al. (2012) in a patient with autism, a nonverbal IQ of 65 and developmental regression (PMID: 23160955). |
OMIM | RCV000032863 | SCV000056632 | pathogenic | Intellectual disability, autosomal dominant 6 | 2012-12-21 | no assertion criteria provided | literature only |