Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Medical Genetics and Genomics, |
RCV001808941 | SCV002058114 | likely pathogenic | Osteogenesis imperfecta, perinatal lethal | 2022-01-11 | no assertion criteria provided | clinical testing | The novel heterozygous mis-sense variant c.1630G>T (p.G544C) has not been observed in gnomAD and 1000g. In-silico bioinformatic software predict this variant by mutation taster as Disease causing and SIFT & PROVEAN as Damaging. Phenotype observed was narrow bell-shaped thorax, bowed long bones and broad metaphyses and diaphysis. Osteogenesis Imperfecta II is an autosomal dominant disorder. Based on the phenotypic observation we classify this variant as likely pathogenic. |