Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
3billion | RCV003152858 | SCV003841327 | pathogenic | Acute infantile liver failure-cerebellar ataxia-peripheral sensory motor neuropathy syndrome | 2023-02-23 | criteria provided, single submitter | clinical testing | The variant is observed at an extremely low frequency in the gnomAD v2.1.1 dataset (total allele frequency: <0.001%). This variant was predicted to alter splicing and result in a loss or disruption of normal protein function. Multiple pathogenic loss-of-function variants are reported downstream of the variant. The variant has been reported to be associated with SCYL1 related disorder (PMID: 32146038). Therefore, this variant is classified as Pathogenic according to the recommendation of ACMG/AMP guideline. |