Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000040854 | SCV000064545 | benign | not specified | 2019-03-13 | criteria provided, single submitter | clinical testing | The p.Arg29905His variant in TTN is classified as benign because it has been identified in 0.13% (40/30508) of South Asian chromosomes and 3 homozygotes by gnomAD (http://gnomad.broadinstitute.org). ACMG/AMP Criteria applied: BA1. |
Gene |
RCV000726674 | SCV000237831 | likely benign | not provided | 2020-12-16 | criteria provided, single submitter | clinical testing | |
Eurofins Ntd Llc |
RCV000726674 | SCV000702070 | uncertain significance | not provided | 2018-08-14 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001088882 | SCV001053375 | likely benign | Dilated cardiomyopathy 1G; Autosomal recessive limb-girdle muscular dystrophy type 2J | 2024-01-04 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV000040854 | SCV003934171 | likely benign | not specified | 2023-05-18 | criteria provided, single submitter | clinical testing | Variant summary: TTN c.89714G>A (p.Arg29905His) results in a non-conservative amino acid change located in the A-band region of the encoded protein sequence. Five of five in-silico tools predict a damaging effect of the variant on protein function. The variant allele was found at a frequency of 0.00017 in 247808 control chromosomes (gnomAD), predominantly at a frequency of 0.0013 within the South Asian subpopulation in the gnomAD database, including 3 homozygotes. The observed variant frequency within South Asian control individuals in the gnomAD database is approximately 3 fold of the estimated maximal expected allele frequency for a pathogenic variant in TTN causing Dilated Cardiomyopathy (0.00039), strongly suggesting that the variant is a benign polymorphism found primarily in populations of South Asian origin. c.89714G>A has been reported in the literature in one individual affected with Hypertrophic Cardiomyopathy without evidence for causality (Burstein_2021). This report does not provide unequivocal conclusions about association of the variant with Dilated Cardiomyopathy. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. The following publication has been ascertained in the context of this evaluation (PMID: 32746448). Four ClinVar submitters have assessed the variant since 2014: one classified the variant as uncertain significance, two as likely benign, and one as benign. Based on the evidence outlined above, the variant was classified as likely benign. |