Total submissions: 7
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Illumina Laboratory Services, |
RCV000677324 | SCV000366293 | benign | ALDH18A1-related de Barsy syndrome | 2018-01-12 | criteria provided, single submitter | clinical testing | This variant was observed in the ICSL laboratory as part of a predisposition screen in an ostensibly healthy population. It had not been previously curated by ICSL or reported in the Human Gene Mutation Database (HGMD: prior to June 1st, 2018), and was therefore a candidate for classification through an automated scoring system. Utilizing variant allele frequency, disease prevalence and penetrance estimates, and inheritance mode, an automated score was calculated to assess if this variant is too frequent to cause the disease. Based on the score and internal cut-off values, a variant classified as benign is not then subjected to further curation. The score for this variant resulted in a classification of benign for this disease. |
Gene |
RCV000442277 | SCV000525843 | benign | not specified | 2016-12-08 | criteria provided, single submitter | clinical testing | This variant is considered likely benign or benign based on one or more of the following criteria: it is a conservative change, it occurs at a poorly conserved position in the protein, it is predicted to be benign by multiple in silico algorithms, and/or has population frequency not consistent with disease. |
Labcorp Genetics |
RCV003765781 | SCV000638201 | benign | Cutis laxa, autosomal dominant 3; Autosomal dominant spastic paraplegia type 9; de Barsy syndrome | 2024-02-01 | criteria provided, single submitter | clinical testing | |
SIB Swiss Institute of Bioinformatics | RCV000677324 | SCV000803546 | benign | ALDH18A1-related de Barsy syndrome | 2018-05-31 | criteria provided, single submitter | curation | This variant is interpreted as a Benign, for Cutis laxa, autosomal recessive, type IIIa, in Autosomal Recessive manner. The following ACMG Tag(s) were applied: BS1 => Allele frequency is greater than expected for disorder. BS2 => Observed in a healthy adult individual for a recessive (homozygous), dominant (heterozygous), or X-linked (hemizygous) disorder, with full penetrance expected at an early age. |
Genome Diagnostics Laboratory, |
RCV001848079 | SCV002106136 | benign | Hereditary spastic paraplegia | 2021-04-15 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV002502200 | SCV002810194 | benign | ALDH18A1-related de Barsy syndrome; Hereditary spastic paraplegia 9A; Autosomal recessive complex spastic paraplegia type 9B; Cutis laxa, autosomal dominant 3 | 2021-08-24 | criteria provided, single submitter | clinical testing | |
Breakthrough Genomics, |
RCV004718360 | SCV005324095 | benign | not provided | criteria provided, single submitter | not provided |