Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Center of Excellence in Genomics and Precision Dentistry, |
RCV001807869 | SCV002058096 | pathogenic | Amelogenesis imperfecta type 1H | criteria provided, single submitter | clinical testing | The heterozygous nonsense mutation c.625G>T p.(Gly209Ter) in ITGB6 (NM_000888.5) was identified in a patient with autosomal recessive amelogenesis imperfecta who also harbored heterozygous splice site variant c.1661-3C>G. A homozygous nonsense mutation in ITGB6 was previously reported in a patient with with hypoplastic amelogenesis imperfecta (Wang el al., 2014). This variant was classified as pathogenic by ACMG guidelines. |