Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV002457717 | SCV002735771 | pathogenic | Cardiovascular phenotype | 2017-11-17 | criteria provided, single submitter | clinical testing | The p.C788* variant (also known as c.2364C>A), located in coding exon 24 of the MYBPC3 gene, results from a C to A substitution at nucleotide position 2364. This changes the amino acid from a cysteine to a stop codon within coding exon 24. This alteration has been reported in one subject with hypertrophic cardiomyopathy (HCM) who also carried a missense alteration in MYH7 (Maron BJ et al. Heart Rhythm, 2012 Jan;9:57-63). In addition to the clinical data presented in the literature, this alteration is expected to result in loss of function by premature protein truncation or nonsense-mediated mRNA decay. As such, this alteration is interpreted as a disease-causing mutation. |