Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV003319215 | SCV004023283 | uncertain significance | Mitochondrial disease | 2023-07-24 | reviewed by expert panel | curation | The m.7724A>T variant in MT-CO2 was reviewed by the Mitochondrial Disease Nuclear and Mitochondrial Variant Curation Expert Panel on July 24, 2023. There are no individuals or families with this variant reported in the medical literature to our knowledge. There are several occurrences in population databases. This variant is present in 0.031% of individuals in GenBank MITOMAP sequences, in 0.039% of individuals in gnomAD v3.1.2 (homoplasmic in all individuals), and in 0.088% of individuals in the Helix dataset (homoplasmic in all individuals). The computational predictor APOGEE gives a consensus rating of neutral with a score of 0.3 (Min=0, Max=1), which predicts no damaging effect on gene function (BP4). There are no cybrid, single fiber, or other studies reported for this variant. In summary, this variant meets criteria to be classified as uncertain significance for primary mitochondrial disease inherited in a mitochondrial manner. This classification was approved by the NICHD/NINDS U24 ClinGen Mitochondrial Disease Variant Curation Expert Panel on July 24, 2023. Mitochondrial DNA-specific ACMG/AMP criteria applied (PMID: 32906214): BP4. |
Wong Mito Lab, |
RCV000854094 | SCV000997127 | benign | Leigh syndrome | 2019-10-17 | criteria provided, single submitter | clinical testing | The NC_012920.1:m.7724A>T (YP_003024029.1:p.Thr47Ser) variant in MTCO2 gene is interpretated to be a Benign variant based on the modified ACMG guidelines (unpublished). This variant meets the following evidence codes: BS1, BS2, BP4 |
Athena Diagnostics | RCV000992350 | SCV001144572 | uncertain significance | not provided | 2018-12-10 | criteria provided, single submitter | clinical testing |