Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Broad Center for Mendelian Genomics, |
RCV000785900 | SCV000924476 | uncertain significance | Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type a, 11 | 2018-06-15 | criteria provided, single submitter | research | The homozygous p.Ala272Val variant was identified by our study in two siblings with muscular dystrophy-dystroglycanopathy. This variant was absent from large population studies. The Alanine (Ala) at position 272 is highly conserved in mammals and evolutionarily distant species, raising the possibility that a change at this position may not be tolerated. Computational prediction tools do not provide strong support for or against an impact to the protein. In summary, the clinical significance of this variant is uncertain. |