*Review* **Overview of Mitochondrial E3 Ubiquitin Ligase MITOL**/**MARCH5 from Molecular Mechanisms to Diseases**

### **Isshin Shiiba 1,2, Keisuke Takeda 1, Shun Nagashima 1 and Shigeru Yanagi 1,2,\***


Received: 3 May 2020; Accepted: 26 May 2020; Published: 27 May 2020

**Abstract:** The molecular pathology of diseases seen from the mitochondrial axis has become more complex with the progression of research. A variety of factors, including the failure of mitochondrial dynamics and quality control, have made it extremely di fficult to narrow down drug discovery targets. We have identified MITOL (mitochondrial ubiquitin ligase: also known as MARCH5) localized on the mitochondrial outer membrane and previously reported that it is an important regulator of mitochondrial dynamics and mitochondrial quality control. In this review, we describe the pathological aspects of MITOL revealed through functional analysis and its potential as a drug discovery target.

**Keywords:** mitochondria; E3 ubiquitin ligase; MITOL/MARCH5
