Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Medical Genetics and Genomics, |
RCV003335982 | SCV004042841 | likely pathogenic | Mitochondrial DNA depletion syndrome 3 (hepatocerebral type) | 2023-10-19 | criteria provided, single submitter | clinical testing | This homozygous intron variant identified in a 38days male patient with liver failure, raised AFP and lactate. This nucleotide change is absent from gnomAD population database [PM2]. In-silico predictions (dbscSNV) favor a deleterious nature of this variant [PP3]. Parental segregation confirmed heterozygous state based on TRIO Exome sequencing. Based on the available evidence, variant has been classified as a "Likely Pathogenic". |