Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV002268270 | SCV002550877 | likely pathogenic | Spinocerebellar ataxia type 29 | 2022-07-26 | criteria provided, single submitter | clinical testing | The variant c.4261G>A (p.(Val1421Met)) in exon 33 of the ITPR1-gene is not found in the gnomAD database, it affects a highly conserved nucleotide, a highly conserved amino acid and there is a small physicochemical difference between Val and Met. This variant has a pathogenic computational verdict based on in silico predictions algorithms. It was found to be de novo in our patient. ACMG criteria used for classification: PM2, PM6, PP2, BP4. |
Genome |
RCV002311994 | SCV000840339 | not provided | Spinocerebellar ataxia type 29; Spinocerebellar ataxia type 15/16; Gillespie syndrome | no assertion provided | phenotyping only | GenomeConnect assertions are reported exactly as they appear on the patient-provided report from the testing laboratory. GenomeConnect staff make no attempt to reinterpret the clinical significance of the variant. |