Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Molecular Genetics, |
RCV003994690 | SCV004812653 | uncertain significance | KBG syndrome | 2024-02-05 | criteria provided, single submitter | clinical testing | This sequence change in ANKRD11 is predicted to replace valine with aspartic acid at codon 467, p.(Val467Asp). The valine residue is highly conserved (100 vertebrates, Multiz Alignments). There is a large physicochemical difference between valine and aspartic acid. The highest population minor allele frequency in the population database gnomAD v4.0 is 0.0005% (6/1,112,004 alleles) in the European (non-Finnish) population. To our knowledge, this variant has not been previously reported in the relevant scientific literature or databases. Computational evidence is uninformative for the missense substitution (REVEL = 0.356). Based on the classification scheme RMH Modified ACMG Guidelines v1.6.1, this variant is classified as a VARIANT OF UNCERTAIN SIGNIFICANCE. Following criteria are met: none. |