Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Foundation for Research in Genetics and Endocrinology, |
RCV000656734 | SCV000778311 | likely pathogenic | KBG syndrome | 2018-04-06 | no assertion criteria provided | clinical testing | The observed variant c.3706C>T (p.Gln1236Ter) is not reported in 1000 Genomes and ExAC databases. The in silico prediction is disease causing by MutationTaster2. |
The Purple Gene Clinic, |
RCV000656734 | SCV005045666 | likely pathogenic | KBG syndrome | 2024-05-29 | no assertion criteria provided | clinical testing | This sequence change creates a stop codon and results in premature protein truncation in the ANKRD11 gene. This variant is absent in gnomAD database. This variant has not been reported in the literature in individuals with ANKRD11-related conditions. Loss-of-function variants in ANKRD11 are known to be pathogenic (PMID: 38515699, 37800809, 37226940). For these reasons, this variant has been classified as likely pathogenic. |