Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000189570 | SCV000243213 | uncertain significance | not provided | 2013-06-17 | criteria provided, single submitter | clinical testing | p.Ala251Thr (GCC>ACC): c.751 G>A in exon 7 of the SRPX2 gene (NM_014467.2) The Ala251Thr missense change has not been published as a mutation, nor has it been reported as a benign polymorphism to our knowledge. It was not observed in approximately 5,300 individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, indicating it is not a common benign variant in these populations. The amino acid substitution is non-conservative, as a non-polar Alanine residue is replaced by a polar Threonine residue. It alters a highly conserved position in the HYR domain, and multiple in silico algorithms predict that Ala251Thr may be damaging to protein structure/function. However, missense mutations have not been reported previously in this region of the protein. Therefore, based on the currently available information, it is unclear whether Ala251Thr is a disease-causing mutation or a rare benign variant. The variant is found in EPILEPSY panel(s). |