Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000200121 | SCV000251577 | uncertain significance | not provided | 2014-07-24 | criteria provided, single submitter | clinical testing | p.Ile410Lys (ATA>AAA): c.1229 T>A in exon 14 of the GFM2 gene (NM_032380.3). The I410K variant hasn't been published as a mutation or reported as a benign polymorphism to our knowledge. The I410K variant is a non-conservative amino acid substitution, which is likely to impact secondary protein structure as these residues differ in polarity, charge, size and/or other properties. The I410K substitution occurs at a position where amino acids with similar properties as Isoleucine are conserved across species. In silico analysis predicts that the I410K variant is probably damaging to the protein structure/function. Therefore, based on the currently available information, it is unclear whether I410K is a pathogenic mutation or a rare benign variant. The variant is found in LSME-MITOP panel(s). |
Genome |
RCV001824675 | SCV002075231 | not provided | Hepatoencephalopathy due to combined oxidative phosphorylation defect type 1; Combined oxidative phosphorylation deficiency 39 | no assertion provided | phenotyping only | Variant classified as Uncertain significance and reported on 08-12-2014 by Lab or GTR ID 26957. GenomeConnect assertions are reported exactly as they appear on the patient-provided report from the testing laboratory. GenomeConnect staff make no attempt to reinterpret the clinical significance of the variant. |