Fig. 2: Reverse transcription polymerase chain reaction (RT-PCR) analysis in patient 078.

RT-PCR analysis across exons 83 and 84 of SYNE1 demonstrated aberrant splicing with the c.16024-13C>G variant being used as a cryptic splice acceptor site. Both the normal and aberrant splice product was observed in patient 078 compared with only a normal splice product in the normal control sample. Aberrant splicing at the c.16024-13C>G site results in the creation of a truncating TGA stop codon immediately after the splice site