Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV003338384 | SCV004048075 | uncertain significance | Stickler syndrome, type I, nonsyndromic ocular | criteria provided, single submitter | clinical testing | The frameshift deletion p.G1446Afs*25 in COL2A1 (NM_001844.5) has been reported to ClinVar as Pathogenic. The p.G1446Afs*25 variant is novel (not in any individuals) in gnomAD Exomes and is novel (not in any individuals) in 1000 Genomes. The frame shifted sequence continues 25 residues until a stop codon is reached. The p.G1446Afs*25 variant is a loss of function variant in the gene COL2A1, which is intolerant of Loss of Function variants. Since, the variant is present in last exon, functional studies will be required to prove protein truncation. For these reasons, this variant has been classified as Uncertain Significance. | |
OMIM | RCV000018933 | SCV000039219 | pathogenic | Spondyloperipheral dysplasia | 2004-08-30 | no assertion criteria provided | literature only |