Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000534336 | SCV000640230 | pathogenic | Charcot-Marie-Tooth disease dominant intermediate B | 2020-10-06 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Benign"; Align-GVGD: "Class C35"). This variant has been observed in several individuals affected with centronuclear myopathy including at least one individual in whom the variant was observed to be de novo (PMID: 19932619, Invitae). This variant is not present in population databases (ExAC no frequency). This sequence change replaces alanine with aspartic acid at codon 618 of the DNM2 protein (p.Ala618Asp). The alanine residue is highly conserved and there is a moderate physicochemical difference between alanine and aspartic acid. |
Laboratory of Medical Genetics, |
RCV001729629 | SCV001976988 | pathogenic | Autosomal dominant centronuclear myopathy | 2021-10-01 | criteria provided, single submitter | clinical testing | PS2, PM1, PM2, PM5, PP2, PP3, PP5 |
Inherited Neuropathy Consortium Ii, |
RCV000534336 | SCV004174762 | uncertain significance | Charcot-Marie-Tooth disease dominant intermediate B | 2016-01-06 | no assertion criteria provided | literature only |