Fig. 7.

Loss of telethonin in A178D heart and partical restoration upon proteasomal inhibition. Top—Cryo-sections of hearts from WT mice, stained for telethonin and titin Z-disc epitope T12; nuclei are visualised with DAPI. Telethonin is localised at the Z-discs. Middle—same staining in A178D hearts treated with vehicle (10% dimethyl sulfoxide in 0.9% saline). Telethonin signal is lost from the Z-discs. Asterisks indicate non-specific staining of the telethonin antibody at the extracellular matrix. Bottom—A178D hearts treated with epoxomicin (0.5 mg kg⁻¹ bodyweight per day for 7 days) show partial restoration of telethonin signal at the Z-disc (arrowheads). Scale bar represents 25 microns. All mice in this experiment are male (n = 2 per group; WT age 116 days, A178D age 115 ± 2 days)