Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002907699 | SCV003244440 | uncertain significance | not provided | 2025-02-04 | criteria provided, single submitter | clinical testing | This sequence change affects codon 112 of the MBD4 mRNA. It is a 'silent' change, meaning that it does not change the encoded amino acid sequence of the MBD4 protein. It affects a nucleotide within the consensus splice site. This variant is present in population databases (rs61753468, gnomAD 0.09%), and has an allele count higher than expected for a pathogenic variant. This variant has not been reported in the literature in individuals affected with MBD4-related conditions. ClinVar contains an entry for this variant (Variation ID: 2038734). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant is not likely to affect RNA splicing. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
Ce |
RCV002907699 | SCV004155503 | likely benign | not provided | 2024-03-01 | criteria provided, single submitter | clinical testing | MBD4: BP4, BP7 |
Ambry Genetics | RCV004983167 | SCV005618021 | uncertain significance | Inborn genetic diseases | 2024-12-04 | criteria provided, single submitter | clinical testing | The c.336C>T variant (also known as p.S112S), located in coding exon 3 of the MBD4 gene, results from a C to T substitution at nucleotide position 336. This nucleotide substitution does not change the amino acid at codon 112. This variant impacts the first base pair of coding exon 3. This nucleotide position is well conserved in available vertebrate species. In silico splice site analysis for this alteration is inconclusive. Based on the available evidence, the clinical significance of this variant remains unclear. |
Prevention |
RCV003961152 | SCV004770698 | likely benign | MBD4-related disorder | 2019-02-28 | no assertion criteria provided | clinical testing | This variant is classified as likely benign based on ACMG/AMP sequence variant interpretation guidelines (Richards et al. 2015 PMID: 25741868, with internal and published modifications). |