A novel TPM2 gene splice-site mutation causes severe congenital myopathy with arthrogryposis and dysmorphic features

Journal of Applied Genetics - Tập 58 - Trang 199-203 - 2016
Magdalena Mroczek1, Dagmara Kabzińska1, Krystyna H. Chrzanowska2, Maciej Pronicki3, Andrzej Kochański1
1Neuromuscular Unit, Mossakowski Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland
2Department of Medical Genetics, The Children’s Memorial Health Institute, Warsaw, Poland
3Department of Pathology, The Children’s Memorial Health Institute, Warsaw, Poland

Tóm tắt

To date, only two splice-site mutations within the TPM2 gene have been shown to be causative for congenital myopathies. While the majority of TPM2 gene mutations are causative for nemaline myopathy, cap disease or distal arthrogryposis, some mutations in this gene have been found to be associated with non-specific congenital myopathy. We report on a patient with such an unspecified congenital myopathy associated with distinctive facial dysmorphic features and distal arthrogryposis. Using the whole exome sequencing (WES) approach we were able to identify a novel heterozygous splice-site mutation within the TPM2 gene, showing the utility of WES in molecular diagnostics of congenital myopathies without recognizable morphological hallmarks.

Tài liệu tham khảo

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