Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV001254936 | SCV001365471 | pathogenic | Lissencephaly due to TUBA1A mutation | 2020-06-29 | criteria provided, single submitter | clinical testing | The variant c.545T>C (p.(Val182Ala)) in the TUBA1A-gene was found to be de novo, it affects a highly conserved nucleotide and a highly conserved amino acid and is located within a functional protein domain. Also it is absent from common databases as gnomAD or ExAC. A different sequence change (c.544G>T), which affects the same codon as the c.545T>C mutation but leads to a different amino acid exchange (p.(Val182APhe)), has already been described as pathogenic in affected individuals (Hebebrand et al. (2019), PMID: 30744660). ACMG criteria used for classification: PS2, PM1, PM2, PM5, PP2 |