Limb and CNS expressed 1 (LIX1)

Limb and CNS expressed 1 (LIX1) is a cytoplasmic and mitochondrial protein encoded by the LIX1 gene in humans, highly conserved across vertebrates[1][4]. LIX1 is primarily recognized as a marker of immature digestive mesenchyme and plays a regulatory role in mitochondrial morphology and respiration[1]. It is a S-palmitoylated protein that localizes to the outer mitochondrial membrane, where it influences the stability of mitochondrial proteins involved in cristae structure (PHB2 and OPA1) and mitochondrial redox signaling[1]. LIX1 modulates the fate and proliferation of digestive mesenchymal progenitor cells through its effects on mitochondrial metabolism and YAP1/TAZ signaling, and is critical for cell lineage decisions[1]. High expression of LIX1 has been associated with poor prognosis and increased cell proliferation in gastrointestinal stromal tumors (GISTs), where it regulates KIT protein level and drives tumorigenesis in part through YAP1/TAZ-mediated pathways[1]. Human LIX1 is also implicated in the rare metabolic disorders 3-Methylglutaconic Aciduria types IV and I[4]. The gene has several aliases including C5orf11 and Lowfat homolog, but it is distinct from its paralog LIX1L[3][4]. There are no known drugs directly targeting LIX1 or described mechanisms of drug action, nor are specific safety concerns reported in the literature[1][4].

Other names

Limb expression 1 homologProtein limb expression 1 homologC5orf11LftLowfat homolog (Drosophila)Lix1 homologLimb expression 1