Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000196151 | SCV000252182 | likely pathogenic | not provided | 2013-01-25 | criteria provided, single submitter | clinical testing | p.Arg258Cys (CGT>TGT): c.772 C>T in exon 10 of the RARS2 gene (NM_020320.3) A R258C missense change that is likely pathogenic was identified in the RARS2 gene. It has not been published as a mutation, nor has it been reported as a benign polymorphism to our knowledge. The amino acid change is non-conservative in that a positively charged Arginine residue is replaced by an uncharged Cysteine residue. This change occurs at a position in the RARS2 protein that is conserved in mammals and multiple in-silico analysis programs predict that R258C is damaging to the RARS2 protein. Therefore, R258C is a strong candidate for a disease-causing mutation, however the possibility that it is a benign variant cannot be excluded. The variant is found in MITONUC-MITOP panel(s). |
Palindrome, |
RCV004577946 | SCV005062031 | pathogenic | Pontocerebellar hypoplasia type 6 | 2024-06-07 | criteria provided, single submitter | clinical testing |