Total submissions: 3
| Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
|---|---|---|---|---|---|---|---|---|
| Gene |
RCV000431043 | SCV000516762 | pathogenic | not provided | 2024-01-16 | criteria provided, single submitter | clinical testing | Reported in two patients in published literature, one of whom met diagnostic criteria for Rubinstein-Taybi syndrome and the other of whom had multiple congenital anomalies and dysmorphic features, but detailed clinical information and familial segregation information were not provided (PMID: 18792986, 35616356); Nonsense variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 25525159, 35616356, 18792986) |
| Labcorp Genetics |
RCV001851047 | SCV002238401 | pathogenic | Rubinstein-Taybi syndrome | 2024-04-10 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg768*) in the CREBBP gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in CREBBP are known to be pathogenic (PMID: 17052327, 18792986). This variant is not present in population databases (gnomAD no frequency). This premature translational stop signal has been observed in individual(s) with Rubinstein-Taybi syndrome (PMID: 18792986). ClinVar contains an entry for this variant (Variation ID: 379578). For these reasons, this variant has been classified as Pathogenic. |
| Clinical Genetics Laboratory, |
RCV005238988 | SCV005882892 | pathogenic | Congenital heart anomalies | 2024-08-22 | criteria provided, single submitter | clinical testing | PVS1, PS2, PS4_Moderate, PM2 |