Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000658304 | SCV000780076 | pathogenic | not provided | 2018-05-15 | criteria provided, single submitter | clinical testing | The c.2943+1_2943+2delGT splice site variant in the COL1A2 gene destroys the canonical splice donor site in intron 44 and is predicted to cause abnormal gene splicing. The adjacent exon 44 is in-frame and encodes a portion of the triple helical domain. Variants resulting in a loss or alteration of this region result in poor winding of the collagen triple helix and a less functional protein. The c.2943+1_2943+2delGT variant is not observed in large population cohorts (Lek et al., 2016). |
Mendelics | RCV002249384 | SCV002518741 | pathogenic | Osteogenesis imperfecta, perinatal lethal | 2022-05-04 | criteria provided, single submitter | clinical testing |