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Available structures
PDB Ortholog search: PDBe RCSB
Aliases LHX3, CPHD3, LIM3, M2-LIM homeobox 3
External IDs MGI: 102673 HomoloGene: 7814 GeneCards: LHX3
RNA expression pattern
PBB GE LHX3 221670 s at fs.png
More reference expression data
Species Human Mouse
RefSeq (mRNA)



RefSeq (protein)



Location (UCSC) Chr 9: 136.2 – 136.21 Mb Chr 2: 26.2 – 26.21 Mb
PubMed search [1] [2]
View/Edit Human View/Edit Mouse

LIM/homeobox protein Lhx3 is a protein that in humans is encoded by the LHX3 gene.[3][4][5]


LHX3 encodes a protein of a large protein family, members of which carry the LIM domain, a unique cysteine-rich zinc-binding domain. The encoded protein is a transcription factor that is required for pituitary development and motor neuron specification. Two transcript variants encoding distinct isoforms have been identified for this gene.[5]

Clinical significance[edit]

Mutations in this gene have been associated with a syndrome of combined pituitary hormone deficiency and rigid cervical spine.[5]


LHX3 has been shown to interact with Ldb1.[6]


  1. ^ "Human PubMed Reference:". 
  2. ^ "Mouse PubMed Reference:". 
  3. ^ Sloop KW, Meier BC, Bridwell JL, Parker GE, Schiller AM, Rhodes SJ (Jan 2000). "Differential activation of pituitary hormone genes by human Lhx3 isoforms with distinct DNA binding properties". Mol Endocrinol. 13 (12): 2212–25. doi:10.1210/me.13.12.2212. PMID 10598593. 
  4. ^ Sloop KW, Showalter AD, Von Kap-Herr C, Pettenati MJ, Rhodes SJ (May 2000). "Analysis of the human LHX3 neuroendocrine transcription factor gene and mapping to the subtelomeric region of chromosome 9". Gene. 245 (2): 237–43. doi:10.1016/S0378-1119(00)00025-1. PMID 10717474. 
  5. ^ a b c "Entrez Gene: LHX3 LIM homeobox 3". 
  6. ^ Jurata LW, Pfaff SL, Gill GN (February 1998). "The nuclear LIM domain interactor NLI mediates homo- and heterodimerization of LIM domain transcription factors". J. Biol. Chem. 273 (6): 3152–7. doi:10.1074/jbc.273.6.3152. PMID 9452425. 

Further reading[edit]

  • Zhadanov AB, Bertuzzi S, Taira M, et al. (1995). "Expression pattern of the murine LIM class homeobox gene Lhx3 in subsets of neural and neuroendocrine tissues". Dev. Dyn. 202 (4): 354–64. doi:10.1002/aja.1002020405. PMID 7626792. 
  • Zhadanov AB, Copeland NG, Gilbert DJ, et al. (1995). "Genomic structure and chromosomal localization of the mouse LIM/homeobox gene Lhx3". Genomics. 27 (1): 27–32. doi:10.1006/geno.1995.1004. PMID 7665181. 
  • Jurata LW, Pfaff SL, Gill GN (1998). "The nuclear LIM domain interactor NLI mediates homo- and heterodimerization of LIM domain transcription factors". J. Biol. Chem. 273 (6): 3152–7. doi:10.1074/jbc.273.6.3152. PMID 9452425. 
  • Bach I, Rodriguez-Esteban C, Carrière C, et al. (1999). "RLIM inhibits functional activity of LIM homeodomain transcription factors via recruitment of the histone deacetylase complex". Nat. Genet. 22 (4): 394–9. doi:10.1038/11970. PMID 10431247. 
  • Glenn DJ, Maurer RA (2000). "MRG1 binds to the LIM domain of Lhx2 and may function as a coactivator to stimulate glycoprotein hormone alpha-subunit gene expression". J. Biol. Chem. 274 (51): 36159–67. doi:10.1074/jbc.274.51.36159. PMID 10593900. 
  • Howard PW, Maurer RA (2000). "Identification of a conserved protein that interacts with specific LIM homeodomain transcription factors". J. Biol. Chem. 275 (18): 13336–42. doi:10.1074/jbc.275.18.13336. PMID 10788441. 
  • Parker GE, Sandoval RM, Feister HA, et al. (2000). "The homeodomain coordinates nuclear entry of the Lhx3 neuroendocrine transcription factor and association with the nuclear matrix". J. Biol. Chem. 275 (31): 23891–8. doi:10.1074/jbc.M000377200. PMID 10818088. 
  • Netchine I, Sobrier ML, Krude H, et al. (2000). "Mutations in LHX3 result in a new syndrome revealed by combined pituitary hormone deficiency". Nat. Genet. 25 (2): 182–6. doi:10.1038/76041. PMID 10835633. 
  • Schmitt S, Biason-Lauber A, Betts D, Schoenle EJ (2000). "Genomic structure, chromosomal localization, and expression pattern of the human LIM-homeobox3 (LHX 3) gene". Biochem. Biophys. Res. Commun. 274 (1): 49–56. doi:10.1006/bbrc.2000.3038. PMID 10903894. 
  • Sloop KW, Dwyer CJ, Rhodes SJ (2001). "An isoform-specific inhibitory domain regulates the LHX3 LIM homeodomain factor holoprotein and the production of a functional alternate translation form". J. Biol. Chem. 276 (39): 36311–9. doi:10.1074/jbc.M103888200. PMID 11470784. 
  • Ostendorff HP, Peirano RI, Peters MA, et al. (2002). "Ubiquitination-dependent cofactor exchange on LIM homeodomain transcription factors". Nature. 416 (6876): 99–103. doi:10.1038/416099a. PMID 11882901. 
  • Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMC 139241Freely accessible. PMID 12477932. 
  • van Meyel DJ, Thomas JB, Agulnick AD (2003). "Ssdp proteins bind to LIM-interacting co-factors and regulate the activity of LIM-homeodomain protein complexes in vivo". Development. 130 (9): 1915–25. doi:10.1242/dev.00389. PMID 12642495. 
  • Kim SS, Kim Y, Shin YL, et al. (2004). "Clinical characteristics and molecular analysis of PIT1, PROP1,LHX3, and HESX1 in combined pituitary hormone deficiency patients with abnormal pituitary MR imaging". Horm. Res. 60 (6): 277–83. doi:10.1159/000074245. PMID 14646405. 
  • Dattani MT (2004). "Borjeson-Forssman-Lehmann syndrome: a novel pituitary phenotype due to mutation in a novel gene". J. Pediatr. Endocrinol. Metab. 16 (9): 1207–9. doi:10.1515/jpem.2003.16.9.1207. PMID 14714741. 
  • West BE, Parker GE, Savage JJ, et al. (2004). "Regulation of the follicle-stimulating hormone beta gene by the LHX3 LIM-homeodomain transcription factor". Endocrinology. 145 (11): 4866–79. doi:10.1210/en.2004-0598. PMID 15271874. 
  • Parker GE, West BE, Witzmann FA, Rhodes SJ (2005). "Serine/threonine/tyrosine phosphorylation of the LHX3 LIM-homeodomain transcription factor". J. Cell. Biochem. 94 (1): 67–80. doi:10.1002/jcb.20287. PMID 15517599. 
  • Sobrier ML, Attié-Bitach T, Netchine I, et al. (2006). "Pathophysiology of syndromic combined pituitary hormone deficiency due to a LHX3 defect in light of LHX3 and LHX4 expression during early human development". Gene Expr. Patterns. 5 (2): 279–84. doi:10.1016/j.modgep.2004.07.003. PMID 15567726. 

External links[edit]

This article incorporates text from the United States National Library of Medicine, which is in the public domain.