CHCHD3 (coiled-coil-helix-coiled-coil-helix domain containing 3) is a peripheral protein of the mitochondrial inner membrane that faces the intermembrane space and serves as a crucial component of the MICOS (mitochondrial contact site and cristae organizing system) complex 1. The protein functions as a scaffolding protein that stabilizes protein complexes involved in maintaining crista architecture and protein import, interacting with mitofilin, OPA1, and Sam50 1. CHCHD3 is essential for maintaining crista integrity, mitochondrial fusion, and overall mitochondrial function 12. Loss of CHCHD3 results in fragmented mitochondria, reduced OPA1 protein levels, impaired mitochondrial fusion, abnormal crista morphology with reduced crista junction opening diameter, and severely restricted oxygen consumption and glycolytic rates 1. The protein undergoes myristoylation at its N-terminus and contains a CHCH domain that is essential for its import and mitochondrial localization 3. Age-related loss of MICOS complex components including CHCHD3 is associated with mitochondrial morphological changes during aging 4. CHCHD3 dysfunction has been implicated in various pathological conditions including congenital heart disease, particularly hypoplastic left heart syndrome 5, and kidney disease through mitochondrial dysfunction in podocytes 6.