LHX1 (LIM homeobox 1) is a sequence-specific DNA-binding transcription factor essential for embryonic development and organogenesis. During early development, LHX1 marks the anterior visceral endoderm and is critical for gastrulation axis formation 1. LHX1 plays a central role in kidney development, where it is expressed in PAX8+ renal vesicles that differentiate into nephron structures including podocytes, proximal tubules, and distal tubules 2. The gene is also vital for embryonic head development, where LHX1 regulates morphogenetic movements through modulation of WNT signaling and transcriptional control of target genes via interactions with transcription factor complexes 3. Clinically, LHX1 dysfunction is implicated in multiple pathologies. Loss-of-function variants in LHX1 and deletions in the 17q12 region (containing LHX1) are associated with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, characterized by uterine and vaginal agenesis 4. In pathological contexts, LHX1 can function as an oncogenic driver; in esophageal squamous cell carcinoma, LHX1 overexpression forms a positive feedback loop with UHRF1 and NKX2-5, promoting tumor growth through epigenetic dysregulation 5. Aberrant LHX1 expression also contributes to preterm birth by inducing endoplasmic reticulum stress via IRE-1/XBP1/CHOP pathway activation 6. Additionally, LHX1-derived long non-coding RNA (LHX1-DT) suppresses renal cell carcinoma progression through miR-590-5p sponging 7. LHX1 is recognized as an oncogenic homeoprotein in medulloblastoma 8.