Respiratory chain inactivation links cartilage-mediated growth retardation to mitochondrial diseases.

ABSTRACT: In childhood, skeletal growth is driven by transient expansion of cartilage in the growth plate. The common belief is that energy production in this hypoxic tissue mainly relies on anaerobic glycolysis and not on mitochondrial respiratory chain (RC) activity. However, children with mitochondrial diseases causing RC dysfunction often present with short stature, which indicates that RC activity may be essential for cartilage-mediated skeletal growth. To elucidate the role of the mitochondrial RC in cartilage growth and pathology, we generated mice with impaired RC function in cartilage. These mice develop normally until birth, but their later growth is retarded. A detailed molecular analysis revealed that metabolic signaling and extracellular matrix formation is disturbed and induces cell de

SUBMITTER: Holzer T 

PROVIDER: S-EPMC6548139 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature