Involved in cristae formation and mitochondrial fusion. Located in mitochondrial inner membrane. Part of MICOS complex. Is expressed in several structures, including adult head; cuboidal/columnar epithelium; embryonic/larval midgut primordium; gut section; and larval muscle cell. Used to study hypoplastic left heart syndrome. Orthologous to several human genes including CHCHD3 (coiled-coil-helix-coiled-coil-helix domain containing 3).
FB Description
Coiled-coil-helix-coiled-coil-helix domain containing 3 (Chchd3) encodes a mitochondrial inner membrane protein that is required for mitochondrial fusion. Loss of Chchd3 leads to inactivation of Hippo activity, decreased tissue growth, cell proliferation defects, oxidative stress and JNK pathway activation.