Abstract: The deubiquitinating enzymes(DUBs) play important roles in a variety of physiological process. The Machado-joseph(MJD) family is one of the six DUBs families. The proteins of maintaining of stability and function of proteins which are clinical factors including Ataxin-3, ATAN3L, JOSD1 and JOSD2, which are widely expressed in various parts of the human body. Among them, Ataxin-3 is also closely related to the MJD disease, a neurodegenerative disease. Recently, the biological function as well as the roles in the progression of diseases of the MJD family members have been extensively studied. This review will summarize these research progress of MJD family members in recent years.