ATAD3B is a human embryonic stem cell specific mitochondrial protein, re-expressed in cancer cells, that functions as dominant negative for the ubiquitous ATAD3A

► ATAD3B is an embryonic pluripotent stem cell specific protein. ► ATAD3B is re-expressed in human cancer cells. ► ATAD3B functions as a dominant negative of ATAD3A. ► ATAD3B negatively regulates the interaction of ATAD3A with matrix nucleoid complexes. ► ATAD3B may function as an adaptor of mitochondrial homeostasis and metabolism.