Complex III assembly factor LYRM7 (LYRM7)

Complex III assembly factor LYRM7 (LYRM7) is a small, nuclear-encoded mitochondrial matrix protein essential for assembly of the mitochondrial respiratory chain complex III (ubiquinol-cytochrome c reductase). It acts as a dedicated chaperone for the Rieske iron-sulfur (Fe-S) protein (UQCRFS1), stabilizing and assisting its maturation and insertion into complex III. LYRM7 binds the co-chaperone HSC20, facilitating iron-sulfur cluster transfer from the main scaffold protein ISCU to UQCRFS1. Loss-of-function mutations in LYRM7 disrupt complex III assembly, resulting in severe mitochondrial complex III deficiency, which clinically manifests as varied early-onset neurometabolic disorders and leukoencephalopathy[1][2][3][4]. To date, there are no known therapeutic drugs that target LYRM7 directly; its dysfunction is primarily characterized by the loss of respiratory chain activity, rather than as a direct drug target.

Other names

LYR motif containing 7LYR motif-containing protein 7C5orf31MZM1LFLJ20796MC3DN8Lyrm7 homolog