Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV001530403 | SCV001739512 | likely pathogenic | Hereditary spastic paraplegia 4 | 2021-07-02 | criteria provided, single submitter | clinical testing | The variant c.314del (p.(Pro105Argfs*56)) in exon 1 of the SPAST gene is not found in the gnomAD database and creates a frame shift starting at codon Pro105. The new reading frame ends in a STOP codon at position 56. Frame shift mutations within this gene are a known mechanism of disease. This variant was found in one of our patients affected by spastic paraplegia. ACMG criteria used for classification: PVS1, PM2. |