Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Lupski Lab, |
RCV000234892 | SCV000291996 | pathogenic | Blepharophimosis; Absent speech; Thick lower lip vermilion; Thin upper lip vermilion; Long eyelashes; Intellectual disability, moderate | 2014-03-27 | criteria provided, single submitter | clinical testing | |
Gene |
RCV000413328 | SCV000491140 | pathogenic | not provided | 2016-11-03 | criteria provided, single submitter | clinical testing | The c.4273dupT pathogenic variant in the ARID1B gene has been reported previously as a de novo variant in an individual with intellectual disability, absent speech, and dysmorphic features (Sim et al., 2014). The duplication causes a frameshift starting with codon Tyrosine 1425, changes this amino acid to a Leucine residue and creates a premature Stop codon at position 34 of the new reading frame, denoted p.Tyr1425LeufsX34. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. It was not observed in approximately 6,500 individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, indicating it is not a common benign variant in these populations. |
Genome- |
RCV001807149 | SCV002054284 | pathogenic | Coffin-Siris syndrome 1 | 2021-07-15 | criteria provided, single submitter | clinical testing |