LHX6 is a LIM homeodomain transcription factor serving as a master regulator of GABAergic interneuron development and specification 1. In the telencephalon, LHX6 acts as a cell fate mediator essential for cortical interneuron specification and migration of GABAergic precursors from the subpallium to the cerebral cortex 1. Beyond neural development, LHX6 functions in craniofacial morphogenesis by negatively regulating the cell cycle inhibitor p57Kip2 to promote palatal cell proliferation 2. LHX6 is regulated by developmental signaling pathways including sonic hedgehog, fibroblast growth factors, and Wnt/β-catenin 1. In the hypothalamus, LHX6 shows divergent functions from telencephalic contexts: it is necessary for neuronal survival and specification of GABAergic subsets through interaction with Dlx1/2, Nkx2-2, and Nkx2-1, but these neurons lack long-distance migration and express distinct neuropeptide profiles 3. Mechanistically, LHX6 regulates mitochondrial homeostasis via PINK1/Parkin-mediated mitophagy and MAPK signaling in palatal mesenchymal cells 4. Clinically, LHX6 functions as a tumor suppressor: its epigenetic downregulation through promoter hypermethylation promotes pancreatic and cervical cancer progression by deregulating tumor-suppressive genes and enhancing proliferative signaling 56. Conversely, LHX6+ interneuron lineages represent cells of origin for FOXR2-driven CNS neuroblastoma 7.