Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV001823457 | SCV002072903 | uncertain significance | Auditory neuropathy-optic atrophy syndrome | criteria provided, single submitter | clinical testing | The missense variant p.R309Q in FDXR (NM_024417.5) has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.R309Q variant is observed in 1/1,07,464 (0.0009%) alleles from individuals of European (Non-Finnish) background in gnomAD Exomes and is novel (not in any individuals) in 1000 Genomes. The p.R309Q missense variant is predicted to be damaging by both SIFT and PolyPhen2. The arginine residue at codon 309 of FDXR is conserved in all mammalian species. The nucleotide c.926 in FDXR is predicted conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Uncertain Significance. |