Perturbed proteins represent tissue markers for LAMA2-related congenital myopathy and—based on information available in uniport—mainly localize to the sarcoplasm, to nuclei, the SR and to mitochondria thus suggesting a global unified organelle vulnerability in LAMA2-mutant skeletal muscle with a predominance to mitochondria. The gene discussed is LAMA2; the disease is congenital myopathy.