Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
UNC Molecular Genetics Laboratory, |
RCV001374603 | SCV001571447 | uncertain significance | Kleefstra syndrome 2 | 2020-04-01 | criteria provided, single submitter | research | The KMT2C c.4918G>C (p.Glu1640Gln) is a missense variant that changes a single amino acid in the encoded protein from glutamic acid to glutamine. This is a rare variant observed in the heterozygous state in 2 out of 251,402 alleles (0.0008%) from unselected individuals in the general population and is not reported in homozygosity. This variant has not been previously reported with disease in the literature. Although nearly all KMT2C variants reported to date in association with its neurodevelopmental disorder are de novo protein truncating variants, the effect of this missense variant on protein function is unknown. This variant has conflicting predictions of pathogenicity by in silico tools and its effect on protein function is unknown. This is considered a variant of uncertain significance. |
Institute of Human Genetics, |
RCV004584438 | SCV002506444 | uncertain significance | See cases | 2021-12-17 | criteria provided, single submitter | clinical testing | ACMG categories: PM2,PP3,BP1 |