MICOS10 is a structural component of the mitochondrial contact site and cristae organizing system (MICOS) complex located on the inner mitochondrial membrane 1. The protein is essential for cristae formation and maintenance of inner membrane architecture, contributing to proper mitochondrial ultrastructure and contact sites with the outer membrane. MICOS10 plays a critical role in mitochondrial respiratory function; loss of MICOS10 expression impairs mitochondrial oxygen consumption, which can be restored through gene overexpression 1. Functionally, MICOS10 is involved in regulating mitochondrial permeability transition pore (mPTP) dynamics, with evidence suggesting it associates with other inner membrane proteins including Got2 and Ndufb7 to modulate calcium handling and energy metabolism 2. Clinically, MICOS10 variants cause hepatocerebral mitochondrial disease characterized by mitochondrial DNA depletion, hepatopathy, and neuropathy, phenotypically similar to MICOS13-associated disease 1. Additionally, MICOS10 expression levels show causal association with colorectal cancer development and demonstrate prognostic significance in cancer outcomes 3. Epigenome-wide studies identify MICOS10 methylation as a novel marker associated with incident type 2 diabetes risk, particularly in Black populations 4.