Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute for Human Genetics and Genomic Medicine, |
RCV002267208 | SCV002549142 | uncertain significance | Cerebellar ataxia-hypogonadism syndrome | 2022-06-07 | no assertion criteria provided | clinical testing | The variant was detected in a patient with clinical suspicion of SCA, family history was negative for a similar condition. The variant is not present in population databases and it has not yet been reported in the literature. The affected amino acid is highly conserved in various species and it affects the IBR domain of the protein where several pathogenic variants have been reported. The patient carries a second variant of unkonwn significance in RNF216, however, no segregation analyses have been performed yet. We therefore consider it a variant of unknown significance. |