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<i>Background and Objectives</i>: Danon disease is a multisystemic disorder associated with variants in the <i>LAMP2</i> gene, mainly affecting the cardiac muscle. Here, we report a multigenerational family from Latvia with two male patients, hemizygous for a novel splice-affecting variant c.928+3A>G. Affected patients exhibit a cardiac phenotype, moderate mental disability, and mild retinal changes. <i>Materials and Methods</i>: Both patients underwent either exome or hypertrophic cardiomyopathy gene panel next-generation sequencing. The pathogenic variant effect was determined using reverse transcription, Sanger sequencing, and high-resolution electrophoresis. <i>Results</i>: Evaluation of the splicing process revealed that approximately 80% of the transcripts exhibited a lack of the entire exon 7. This alteration was predicted to cause a shift of the reading frame, consequently introducing a premature stop codon downstream in the sequence. <i>Conclusions</i>: Based on our data, we propose that c.928+3A>G is a pathogenic variant associated with Danon disease.