Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001203117 | SCV001374264 | pathogenic | Charcot-Marie-Tooth disease type 4 | 2019-08-23 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Loss-of-function variants in MTMR2 are known to be pathogenic (PMID: 10802647). This variant has not been reported in the literature in individuals with MTMR2-related conditions. This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Glu531*) in the MTMR2 gene. It is expected to result in an absent or disrupted protein product. |
Ambry Genetics | RCV003163530 | SCV003887523 | pathogenic | Inborn genetic diseases | 2023-01-06 | criteria provided, single submitter | clinical testing | The c.1591G>T (p.E531*) alteration, located in exon 13 (coding exon 13) of the MTMR2 gene, consists of a G to T substitution at nucleotide position 1591. This changes the amino acid from a glutamic acid (E) to a stop codon at amino acid position 531. This alteration is expected to result in loss of function by premature protein truncation or nonsense-mediated mRNA decay. This variant was not reported in population-based cohorts in the Genome Aggregation Database (gnomAD). Based on the available evidence, this alteration is classified as pathogenic. |