MSTO1 (Misato Mitochondrial Distribution And Morphology Regulator 1) is a Protein Coding gene. The MSTO1 gene, located on 1q22, is conserved in chimpanzee, Rhesus monkey, dog, cow, mouse, and rat, etc. MSTO1 belongs to the misato family. 7 alternatively spliced human isoforms have been reported. MSTO1 encodes a cytosolic mitochondrial fusion protein, misato homolog 1 or MSTO1. Human MSTO1 is involved in the regulation of mitochondrial distribution and morphology and its unregulated expression leads to a mitochondrial disorder. MSTO1 mutations have been shown to cause clinical manifestations suggestive of mitochondrial dysfunction, such as muscle weakness, short stature, motor developmental delay, and cerebellar atrophy. Both autosomal dominant and recessive modes of inheritance have been suggested.