Mitochondriopathies and bone health

Mitochondrial disorders represent a heterogeneous group of clinical syndromes sharing energy deficit of the mitochondrial metabolism.Although still today mitochondrial disease generally means a disorder with the oxidative phosphorylation deficiency, new knowledge of human mitochondrial genetics, biology and pathophysiology suggest the possibility of a broader spectrum of alterations and, accordingly, of presentations/clinical features.In this brief review, we will introduce the concept of why it is appropriate to write about mitochondrial disorders and bone health, passing through general concepts of mitochondrial biology and pathophysiology, because recent evidence connect the mitochondrial function to the physiology of bone cells, especially the bone-resorbing cells named osteoclasts, but not only.A deeper knowledge of mitochondrial pathophysiology in bone cells may provide one fundamental key for understanding the pathogenesis of metabolic pathways underlying skeletal diseases, both in "bone modeling" (childhood, adolescence, young adults) and in "bone remodeling" (adult, post-menopause women, elderly), and especially for the development of new therapeutical options to treat metabolic bone diseases, such as osteoporosis, and also other metabolic bone disorders, reducing the fragility fracture risk.