Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Bioinformatics Research Center, |
RCV001255865 | SCV001190007 | likely pathogenic | Autosomal dominant osteopetrosis 2 | 2020-03-17 | criteria provided, single submitter | clinical testing | The c.T1769G (p.Leu614Arg) is novel and has not been reported in the 1000 Genomes Project build 20130502 (2,504 samples, accessed 9/18/2019), dpSNP build 153 (accessed 9/18/2019), or Exome Aggregation Consortium (ExAC, 60,706 samples, accessed 9/18/2019) database. A different mutation in the same position was reported in ClinVar database (rs1064794323 - Leu614Pro) in a child with severe osteopetrosis, anemia, blindness, neurological impairment and macrocephaly, who died at 4 years of age, and also had a deletion in exon 17 of the CLCN7 gene (Frattini et al., 2003). |