Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000255139 | SCV000321860 | pathogenic | not provided | 2018-07-16 | criteria provided, single submitter | clinical testing | The c.3609+1 G>A splice site variant in the LAMA3 gene has been previously reported in association with JEB (Teebi et al., 2010) and has been observed in the homozygous state in patients referred to GeneDx for testing. This variant destroys the canonical splice donor site in intron 27 and is expected to cause abnormal gene splicing. The variant is not observed in large population cohorts (Lek et al., 2016). In summary, we consider this variant to be pathogenic. |