Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV003340945 | SCV004047823 | uncertain significance | Autosomal recessive limb-girdle muscular dystrophy type 2O | criteria provided, single submitter | clinical testing | The missense c.1742T>C (p.Met581Thr) variant in POMGNT1 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The variant is novel (not in any individuals) in gnomAD Exomes and 1000 Genomes. The amino acid Met at position 581 is changed to a Thr changing protein sequence and it might alter its composition and physico-chemical properties. The amino acid change p.Met581Thr in POMGNT1 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Uncertain significance. |