Patients carrying BVES mutations develop a recessive form of limb-girdle muscular dystrophy (LGMDR25), with most patients also suffering from cardiac arrhythmia (sinus bradycardia, sinus tachycardia, or atrioventricular (AV)-block) [5, 10, 11, 15, 19, 40]. This evidence concerns the gene POPDC1 and limb-girdle muscular dystrophy.