Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV001001484 | SCV001158758 | benign | not specified | 2018-07-09 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001517237 | SCV001725703 | benign | Osteogenesis imperfecta type I | 2025-01-13 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV002497319 | SCV002813137 | likely benign | Osteogenesis imperfecta with normal sclerae, dominant form; Osteogenesis imperfecta, perinatal lethal; Osteogenesis imperfecta type III; Infantile cortical hyperostosis; Ehlers-Danlos syndrome, arthrochalasia type; Osteogenesis imperfecta type I; Osteoporosis; Combined osteogenesis imperfecta and Ehlers-Danlos syndrome 1 | 2022-05-06 | criteria provided, single submitter | clinical testing | |
Breakthrough Genomics, |
RCV004710233 | SCV005249998 | benign | not provided | criteria provided, single submitter | not provided | ||
Women's Health and Genetics/Laboratory Corporation of America, |
RCV001001484 | SCV005726305 | benign | not specified | 2024-11-15 | criteria provided, single submitter | clinical testing | Variant summary: COL1A1 c.104-441G>T, also reported as rs1800012, is located at a position not widely known to affect splicing. Consensus agreement among computation tools predict no significant impact on normal splicing. However, these predictions have yet to be confirmed by functional studies. The variant allele was found at a frequency of 0.14 in 152102 control chromosomes in the gnomAD database, including 1672 homozygotes. The observed variant frequency is approximately 4865.67 fold of the estimated maximal expected allele frequency for a pathogenic variant in COL1A1 causing Osteogenesis Imperfecta phenotype (2.8e-05). To our knowledge, no occurrence of c.104-441G>T in individuals affected with Osteogenesis Imperfecta and no experimental evidence demonstrating its impact on protein function have been reported. ClinVar contains an entry for this variant (Variation ID: 811535). Based on the evidence outlined above, the variant was classified as benign. |