MNX1 is a homeobox transcription factor essential for motor neuron and pancreatic development 1. As a sequence-specific DNA-binding protein, MNX1 recognizes regulatory elements to modulate target gene transcription, including negative regulation of CHX10 and interneuron genes in motor neurons 2. During neurogenesis, MNX1 (also called Hb9) is a canonical motor neuron marker expressed in >95% of differentiated motor neurons 2. In pancreatic development, MNX1 participates in cell fate specification and is preferentially expressed in pancreatic progenitor-subtype pancreatic cancers alongside PDX1 and FOXA2/3 3. MNX1 cooperates with HNF1B to govern genes essential for gastrointestinal stem cells in intraductal papillary mucinous neoplasm (IPMN) lineages 4. Clinically, MNX1 mutations cause Currarino syndrome, a rare autosomal dominant disorder characterized by presacral masses, sacral agenesis, and anorectal anomalies resulting from dorsal-ventral patterning defects 1. However, MNX1 mutations are detected in only 57.4% of Currarino syndrome patients, suggesting additional genetic loci contribute to disease etiology 1. Beyond Mendelian disease, aberrant MNX1 activation occurs in acute myeloid leukemia through enhancer hijacking via deletion 7q, promoting leukemia cell fitness 5. The lncRNA MNX1-AS1 is separately implicated in multiple cancers, correlating with poor prognosis and chemoresistance 6.