Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV003323837 | SCV001442812 | likely pathogenic | Kleefstra syndrome 2 | 2023-07-28 | criteria provided, single submitter | clinical testing | Variant summary: KMT2C c.13334G>A (p.Gly4445Asp) results in a non-conservative amino acid change located in the Extended PHD (ePHD) domain (IPR034732) of the encoded protein sequence. Four of five in-silico tools predict a damaging effect of the variant on protein function. The variant was absent in 250922 control chromosomes. c.13334G>A has been observed in a de novo patient in our laboratory with confirmed maternity and paternity. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. No submitters have cited clinical-significance assessments for this variant to ClinVar after 2014. Based on the evidence outlined above, the variant was classified as likely pathogenic. |