Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Foundation for Research in Genetics and Endocrinology, |
RCV001779965 | SCV001960967 | uncertain significance | Kleefstra syndrome 2 | 2021-02-04 | criteria provided, single submitter | research | A heterozygous missense variation in exon 39 of the KMT2C gene that results in the amino acid substitution of Isoleucine for Methionine at codon 3097 was detected. The observed variant c.9291G>A (p.Met3097Ile) has not been reported in the 1000 genomes and gnomAD databases. The in silico prediction of the variant is damaging by SIFT, LRT and MutationTaster2. The reference codon is conserved across species. Segregation analysis showed the variant to be maternal in origin. In summary, the variant meets our criteria to be classified as a variant of uncertain significance. |