Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
New York Genome Center | RCV001839183 | SCV002099124 | uncertain significance | Neurodevelopmental disorder with hypotonia, neuropathy, and deafness | 2021-04-09 | criteria provided, single submitter | clinical testing | The inherited heterozygous missense variant, c.7228G>C (p.Ala2410Pro) in the SPTBN4 gene has not been reported in affected individuals in the literature. The variant has 0.00003944 allele frequency (6 out of 152,134 heterozygous alleles, no homozygotes) in the gnomAD database indicating it is not a common benign allele in the populations represented in that database. In silico tools provide conflicting predictions about potential pathogenicity of this variant. Based on the available evidence, the inherited c.7228G>C (p.Ala2410Pro) variant in the SPTBN4 gene is reported as a variant of uncertain significance. |