Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Area of Clinical and Molecular Genetics, |
RCV000681525 | SCV000700090 | pathogenic | Short stature-brachydactyly-obesity-global developmental delay syndrome | 2018-03-21 | criteria provided, single submitter | clinical testing | The c.1239_1246dup results in the duplication of 8 nucleotides causing a frame shift at codon 416, that predicts the apparition of a premature stop codon at position 442, which predicts a truncated protein 251 amino acids shorter than the wild type. This variant was detected in an affected individual, that had also the pathogenic variant c.431_432del, p.(Glu144Valfs*3). |
Gene |
RCV004721492 | SCV005327568 | pathogenic | not provided | 2023-09-01 | criteria provided, single submitter | clinical testing | Frameshift 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: 30006058) |