Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratorio de Biología Molecular, |
RCV001849545 | SCV001951007 | pathogenic | Perry syndrome | 2021-08-19 | no assertion criteria provided | clinical testing | The c.200G>A variant in DCTN1 (NM_004082.4) predicts a glycine-to-aspartic acid substitution at codon 67 (p.G67D). This variant was present in two siblings of an Argentine family with Perry Disease and absent in an asymptomatic brother. Ages of onset for affected individuals were: 55 and 56 years. There are two additional families with this variant reported in the literature (Aji et al, 2013; Chung et al, 2014). According to ACMG guidelines (Richards, 2015) this variant can be classified as "pathogenic" (supporting evidence PM1, PM2, PP1, PP3, PP4_strong, PP5). Functional studies were not performed yet. |
Inherited Neuropathy Consortium Ii, |
RCV001849545 | SCV004174454 | uncertain significance | Perry syndrome | 2016-01-06 | no assertion criteria provided | literature only |