Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Department of Neurology, |
RCV001580364 | SCV001786702 | likely pathogenic | X-linked lymphoproliferative disease due to SH2D1A deficiency | no assertion criteria provided | clinical testing | We use extended WES to identify a novel genomic structure variation combined by paracentric inversion and large size deletions of the SH2D1A gene in an XLP1 male patient. The variant functionally disrupted the splice site causing the exon 2 skipping was confirmed by Inversion-PCR, RT-PCR, and Gap-PCR. |