LHX1

Summary

Gene Symbol: LHX1
Description: LIM homeobox 1
Alias: LIM-1, LIM1, LIM/homeobox protein Lhx1, LIM homeobox protein 1, homeobox protein Lim-1
Species: human
Products:     LHX1

Top Publications

  1. Ledig S, Brucker S, Barresi G, Schömburg J, Rall K, Wieacker P. Frame shift mutation of LHX1 is associated with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Hum Reprod. 2012;27:2872-5 pubmed publisher
    ..Recently, in approximately 6% of MRKH patients, deletions of chromosomal region 17q12 have been identified. The LHX1 gene, which is located in the deletion interval, has been suggested to be a strong candidate, because targeting ..
  2. Ledig S, Schippert C, Strick R, Beckmann M, Oppelt P, Wieacker P. Recurrent aberrations identified by array-CGH in patients with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril. 2011;95:1589-94 pubmed publisher
    ..To identify genetic causes of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome...
  3. Bernardini L, Gimelli S, Gervasini C, Carella M, Baban A, Frontino G, et al. Recurrent microdeletion at 17q12 as a cause of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome: two case reports. Orphanet J Rare Dis. 2009;4:25 pubmed publisher
    ..The deletion encompassed two candidate genes, TCF2 and LHX1. Mutational screening of these genes in a selected group of 20 MRKH females without 17q12 deletion was negative...
  4. Hunter C, Rhodes S. LIM-homeodomain genes in mammalian development and human disease. Mol Biol Rep. 2005;32:67-77 pubmed
    ..In this article, we review the roles of the LIM-HD proteins in mammalian development and their involvement in human diseases. ..
  5. Sajan S, Rubenstein J, Warchol M, Lovett M. Identification of direct downstream targets of Dlx5 during early inner ear development. Hum Mol Genet. 2011;20:1262-73 pubmed publisher
    ..confirmed direct binding of Dlx5 to promoters of seven of these (Atbf1, Bmper, Large, Lrrtm1, Msx1, Ebf1 and Lhx1) in a cell line over-expressing Dlx5...
  6. Hiratsuka K, Monkawa T, Akiyama T, Nakatake Y, Oda M, Goparaju S, et al. Induction of human pluripotent stem cells into kidney tissues by synthetic mRNAs encoding transcription factors. Sci Rep. 2019;9:913 pubmed publisher
    ..Subsequently, the second set - HNF1A, GATA3, GATA1 and EMX2, differentiated these cells into PAX8+LHX1+ pretubular aggregates in another 2 days...
  7. Kitagaki J, Ueda Y, Chi X, Sharma N, Elder C, Truffer E, et al. FGF8 is essential for formation of the ductal system in the male reproductive tract. Development. 2011;138:5369-78 pubmed publisher
    ..Mechanistically, FGF8 functions upstream of Lhx1 expression in forming the nephron, and analysis of Fgf8 mutants similarly shows deficient Lhx1 expression in the ..
  8. Pereira G, Malheiros J, Ospina A, Chardulo L, Curi R. Exome sequencing in genomic regions related to racing performance of Quarter Horses. J Appl Genet. 2019;60:79-86 pubmed publisher
    ..ABCG5, COL11A1, GEN1, SOCS3, MICAL1, SPTBN1, EPB41L3, and SHQ1) and nine new candidate genes (AKNA, ARMC2, FKBP15, LHX1, NOL10, TMEM192, ZFP37, FIG4, and HNRNPU), some of them with known function, were related to racing performance in ..
  9. Hamaidi I, Coquard C, Danilin S, Dormoy V, Béraud C, Rothhut S, et al. The Lim1 oncogene as a new therapeutic target for metastatic human renal cell carcinoma. Oncogene. 2019;38:60-72 pubmed publisher
    ..shown that the sonic hedgehog-Gli signaling pathway is oncogenic in CCC allowing us to identify the developmental Lim1 transcription factor as a Gli target and as a new oncogene in CCC regulating cell proliferation and apoptosis, and ..

More Information

Publications94

  1. Winchell C, Jacobs D. Expression of the Lhx genes apterous and lim1 in an errant polychaete: implications for bilaterian appendage evolution, neural development, and muscle diversification. Evodevo. 2013;4:4 pubmed publisher
    ..We expand on that study here by asking whether expression of the LIM homeobox (Lhx) genes apterous and lim1 in the annelid Neanthes arenaceodentata supports homology of the dorsal branches as well as the proximodistal axes ..
  2. Kilpeläinen T, Bentley A, Noordam R, Sung Y, Schwander K, Winkler T, et al. Multi-ancestry study of blood lipid levels identifies four loci interacting with physical activity. Nat Commun. 2019;10:376 pubmed publisher
    ..We find four loci, in/near CLASP1, LHX1, SNTA1, and CNTNAP2, that are associated with circulating lipid levels through interaction with physical activity; ..
  3. Song R, Kidd L, Janssen A, Yosypiv I. Conditional ablation of the prorenin receptor in nephron progenitor cells results in developmental programming of hypertension. Physiol Rep. 2018;6:e13644 pubmed publisher
    ..with expression in CM cells previously shown to direct kidney development, including Notch1, β-catenin, Lef1, Lhx1, Jag1, and p53, were downregulated...
  4. Chiswell B, Stiegler A, Razinia Z, Nalibotski E, Boggon T, Calderwood D. Structural basis of competition between PINCH1 and PINCH2 for binding to the ankyrin repeat domain of integrin-linked kinase. J Struct Biol. 2010;170:157-63 pubmed publisher
    ..Here we report that the LIM1 domains of PINCH1 and PINCH2 directly compete for the same binding site on the ankyrin repeat domain (ARD) of ILK...
  5. Zhang L, Ettou S, Khalid M, Taglienti M, Jain D, Jung Y, et al. EED, a member of the polycomb group, is required for nephron differentiation and the maintenance of nephron progenitor cells. Development. 2018;145: pubmed publisher
    ..i>Lhx1, normally expressed in the renal vesicle, was overexpressed in kidneys of Eed mutant mice...
  6. Chen K, Dong S, Wu N, Wu Z, Zhou Y, Li K, et al. A novel multiplex fluorescent competitive PCR for copy number variation detection. Genomics. 2018;: pubmed publisher
    ..With this method, we tested 21 clinical samples with potential LIM homeobox 1 (LHX1) or T-box 6 (TBX6) deletion...
  7. Espiritu E, Crunk A, Bais A, Hochbaum D, Cervino A, Phua Y, et al. The Lhx1-Ldb1 complex interacts with Furry to regulate microRNA expression during pronephric kidney development. Sci Rep. 2018;8:16029 pubmed publisher
    ..However, how the initial kidney field size is established, patterned, and proportioned is not well characterized. Lhx1 is a transcription factor expressed in pronephric progenitors and is required for specification of the kidney, but ..
  8. Shi W, Xu G, Wang C, Sperber S, Chen Y, Zhou Q, et al. Heat shock 70-kDa protein 5 (Hspa5) is essential for pronephros formation by mediating retinoic acid signaling. J Biol Chem. 2015;290:577-89 pubmed publisher
    ..Hspa5 morphants, pronephros formation was strongly inhibited with the reduction of pronephric marker genes Lim homeobox protein 1 (lhx1), pax2, and β1 subunit of Na/K-ATPase (atp1b1)...
  9. Park K, Gumbiner B. Cadherin-6B is required for the generation of Islet-1-expressing dorsal interneurons. Biochem Biophys Res Commun. 2015;459:504-8 pubmed publisher
    ..Our results thus indicate that Cadherin-6B is necessary for the generation of Islet-1-positive dorsal interneurons, as well as the initiation of pre-migratory neural crest cell emigration. ..
  10. Csont T, Murlasits Z, Ménesi D, Kelemen J, Bencsik P, Pipicz M, et al. Tissue-specific Gene Expression in Rat Hearts and Aortas in a Model of Vascular Nitrate Tolerance. J Cardiovasc Pharmacol. 2015;65:485-93 pubmed publisher
    ..increased: Tas2r119, Ihh, Rrad, Npm1, Snai1; decreased: Tubb2b, Usp15, Sema6c, Wfdc2, Rps21, Ramp2, Galr1, Atxn1, Lhx1) in vascular nitrate tolerance...
  11. Tanigawa S, Wang H, Yang Y, Sharma N, Tarasova N, Ajima R, et al. Wnt4 induces nephronic tubules in metanephric mesenchyme by a non-canonical mechanism. Dev Biol. 2011;352:58-69 pubmed publisher
    ..that Wnt4 protein, which is required for nephron formation, induces tubule formation and differentiation markers Lim1 and E-cadherin in MM cells, but does not activate a TCF reporter or up regulate expression of canonical Wnt target ..
  12. Shirazi Fard S, Thyselius M, All Ericsson C, Hallböök F. The terminal basal mitosis of chicken retinal Lim1 horizontal cells is not sensitive to cisplatin-induced cell cycle arrest. Cell Cycle. 2014;13:3698-706 pubmed publisher
    ..b>Lim1 expressing (+) horizontal progenitor cells (HPCs) have a heterogenic final cell cycle, with some cells undergoing a ..
  13. Nowotschin S, Costello I, Piliszek A, Kwon G, Mao C, Klein W, et al. The T-box transcription factor Eomesodermin is essential for AVE induction in the mouse embryo. Genes Dev. 2013;27:997-1002 pubmed publisher
    ..plays an essential role in AVE recruitment, in part by directly activating the homeobox transcription factor Lhx1. Thus, Eomes function in the visceral endoderm (VE) initiates an instructive transcriptional program controlling AP ..
  14. Liu H, Chen S, Yao X, Li Y, Chen C, Liu J, et al. Histone deacetylases 1 and 2 regulate the transcriptional programs of nephron progenitors and renal vesicles. Development. 2018;145: pubmed publisher
    ..renal vesicles (RVs), Hdac1/2 mutant kidneys lack nascent nephrons or mature glomeruli, a phenocopy of Lhx1 mutants...
  15. Ip C, Fossat N, Jones V, Lamonerie T, Tam P. Head formation: OTX2 regulates Dkk1 and Lhx1 activity in the anterior mesendoderm. Development. 2014;141:3859-67 pubmed publisher
    ..Expression of Dkk1 and Lhx1, two genes that are also essential for head formation, is disrupted in the AME of the conditional Otx2-deficient ..
  16. Kohl A, Marquardt T, Klar A, Sela Donenfeld D. Control of axon guidance and neurotransmitter phenotype of dB1 hindbrain interneurons by Lim-HD code. J Neurosci. 2015;35:2596-611 pubmed publisher
    ..the pancreatic transcription factor 1a (Ptf1a), the homeobox TF-Lbx1 and the Lim-homeodomain (Lim-HD), and TF Lhx1 and Lhx5...
  17. Junttila S, Saarela U, Halt K, Manninen A, Pärssinen H, Lecca M, et al. Functional genetic targeting of embryonic kidney progenitor cells ex vivo. J Am Soc Nephrol. 2015;26:1126-37 pubmed publisher
    ..Moreover, drMM cells transduced with viral vectors mediating Lhx1 knockdown were excluded from the nephric tubules, whereas cells transduced with control vectors were incorporated...
  18. Fossat N, Ip C, Jones V, Studdert J, Khoo P, Lewis S, et al. Context-specific function of the LIM homeobox 1 transcription factor in head formation of the mouse embryo. Development. 2015;142:2069-79 pubmed publisher
    b>Lhx1 encodes a LIM homeobox transcription factor that is expressed in the primitive streak, mesoderm and anterior mesendoderm of the mouse embryo...
  19. Futel M, Leclerc C, Le Bouffant R, Buisson I, Néant I, Umbhauer M, et al. TRPP2-dependent Ca2+ signaling in dorso-lateral mesoderm is required for kidney field establishment in Xenopus. J Cell Sci. 2015;128:888-99 pubmed publisher
    ..Knockdown of pkd2 in the kidney field results in the downregulation of pax8, but not of other kidney field genes (lhx1, osr1 and osr2)...
  20. Zhou F, Gou S, Xiong J, Wu H, Wang C, Liu T. Oncogenicity of LHX2 in pancreatic ductal adenocarcinoma. Mol Biol Rep. 2014;41:8163-7 pubmed publisher
    The LIM proteins (Lhx1, Lhx2, Lhx3 and Lhx4) have been report to play important roles in human development...
  21. Miquelajáuregui A, Varela Echavarría A, Ceci M, García Moreno F, Ricaño I, Hoang K, et al. LIM-homeobox gene Lhx5 is required for normal development of Cajal-Retzius cells. J Neurosci. 2010;30:10551-62 pubmed publisher
    ..Here, we show that two closely related LIM-homeobox genes Lhx1 and Lhx5 are expressed in reelin+ cells in various regions in the mouse telencephalon at or adjacent to sites where ..
  22. Hooker L, Smoczer C, Abbott S, Fakhereddin M, Hudson J, Crawford M. Xenopus pitx3 target genes lhx1 and xnr5 are identified using a novel three-fluor flow cytometry-based analysis of promoter activation and repression. Dev Dyn. 2017;246:657-669 pubmed publisher
    ..We isolated and characterized pitx3 target genes lhx1 and xnr5 using a novel three-fluor flow cytometry tool that was designed to dissect promoters with multiple binding ..
  23. Ren B, Li X, Zhang J, Fan J, Duan J, Chen Y. PDLIM5 mediates PKCε translocation in PMA-induced growth cone collapse. Cell Signal. 2015;27:424-35 pubmed publisher
    ..Furthermore, in neurons, application of PDLIM5 shRNA or over-expression of PDLIM5 LIM1-3 mutants reduced the amount of PKCε in the membrane...
  24. Yu J, Mu J, Guo Q, Yang L, Zhang J, Liu Z, et al. Transcriptomic profile analysis of mouse neural tube development by RNA-Seq. IUBMB Life. 2017;69:706-719 pubmed publisher
    ..mouse neural tube development, including 45 DEGs involved in CNS development, among which Bmp2, Ascl1, Olig2, Lhx1, Wnt7b and Eomes might play the important roles...
  25. Li R, Fu F, Zhang Y, Li D, Liao C. Prenatal diagnosis of 17q12 duplication and deletion syndrome in two fetuses with congenital anomalies. Taiwan J Obstet Gynecol. 2014;53:579-82 pubmed publisher
    ..93-Mb deletion of 17q12 in Fetus 2. Both the duplicated and deleted regions included the HNF1B and LHX1 genes. Neither the duplication nor deletion was inherited from the parents...
  26. Rahman M, Spitzhorn L, Wruck W, Hagenbeck C, Balan P, Graffmann N, et al. The presence of human mesenchymal stem cells of renal origin in amniotic fluid increases with gestational time. Stem Cell Res Ther. 2018;9:113 pubmed publisher
    ..expressed the master renal progenitor markers SIX2 and CITED1, in addition to typical renal proteins such as PODXL, LHX1, BRN1 and PAX8. Albumin endocytosis assays demonstrated the functionality of AF-MSCs as renal cells...
  27. Klimova L, Antosova B, Kuzelova A, Strnad H, Kozmik Z. Onecut1 and Onecut2 transcription factors operate downstream of Pax6 to regulate horizontal cell development. Dev Biol. 2015;402:48-60 pubmed publisher
    ..Analysis of genes involved in the horizontal cell genesis such as Foxn4, Ptf1a, Prox1 and Lim1 showed that although horizontal cells are initially formed, they are not maintained in Onecut-deficient retinae...
  28. Rotti H, Mallya S, Kabekkodu S, Chakrabarty S, Bhale S, Bharadwaj R, et al. DNA methylation analysis of phenotype specific stratified Indian population. J Transl Med. 2015;13:151 pubmed publisher
    ..Bisulfite DNA sequencing of prakriti specific multiple CpG sites in promoters and 5'-UTR such as; LHX1 (Vata prakriti), SOX11 (Pitta prakriti) and CDH22 (Kapha prakriti) were validated...
  29. Chiga M, Ohmori T, Ohba T, Katabuchi H, Nishinakamura R. Preformed Wolffian duct regulates Müllerian duct elongation independently of canonical Wnt signaling or Lhx1 expression. Int J Dev Biol. 2014;58:663-8 pubmed publisher
    ..a few reports supported this notion in mice, including studies on Wnt9b mutant mice and Wolffian duct-specific Lhx1 deletion. However, anatomical ablation of the Wolffian duct has not been established in mice...
  30. Buisson I, Le Bouffant R, Futel M, Riou J, Umbhauer M. Pax8 and Pax2 are specifically required at different steps of Xenopus pronephros development. Dev Biol. 2015;397:175-90 pubmed publisher
    ..Neither Pax2 nor Pax8 is essential to glomus development. We further show that Pax8 controls hnf1b, but not lhx1 and Osr2, expression in the kidney field as soon as the mid-neurula stage...
  31. Zhu Q, High F, Zhang C, Cerveira E, Russell M, Longoni M, et al. Systematic analysis of copy number variation associated with congenital diaphragmatic hernia. Proc Natl Acad Sci U S A. 2018;115:5247-5252 pubmed publisher
    ..These CDH-associated CNVs reveal high-priority candidate genes including HLX, LHX1, and HNF1B We also discuss CNVs that are present in only one patient in the cohort but have additional ..
  32. Morizane R, Lam A, Freedman B, Kishi S, Valerius M, Bonventre J. Nephron organoids derived from human pluripotent stem cells model kidney development and injury. Nat Biotechnol. 2015;33:1193-200 pubmed
    ..The NPCs possess the developmental potential of their in vivo counterparts and form PAX8+ LHX1+ renal vesicles that self-organize into nephron structures...
  33. Zhang J, Cao H, Xie J, Fan C, Xie Y, He X, et al. The oncogene Etv5 promotes MET in somatic reprogramming and orchestrates epiblast/primitive endoderm specification during mESCs differentiation. Cell Death Dis. 2018;9:224 pubmed publisher
    ..In this study, we screened a pool of SSCs TFs (Bcl6b, Lhx1, Foxo1, Plzf, Id4, Taf4b, and Etv5), and found that oncogene Etv5 could dramatically increase the efficiency of ..
  34. Abbasi N, Hashemi S, Salehi M, Jahani H, Mowla S, Soleimani M, et al. Influence of oriented nanofibrous PCL scaffolds on quantitative gene expression during neural differentiation of mouse embryonic stem cells. J Biomed Mater Res A. 2016;104:155-64 pubmed publisher
    ..Analysis also showed higher expression of dorso-ventral neural markers (Isl1/2 and Lim1/2) than motor neuron progenitor markers (Pax6, Nkx6.1, and olig2) in aligned nanofibers than in the T group...
  35. Bedont J, Legates T, Buhr E, Bathini A, Ling J, Bell B, et al. An LHX1-Regulated Transcriptional Network Controls Sleep/Wake Coupling and Thermal Resistance of the Central Circadian Clockworks. Curr Biol. 2017;27:128-136 pubmed publisher
    ..The transcription factor LHX1 drives SCN Vip expression, and cellular desynchrony in Lhx1-deficient SCN largely results from Vip loss [17, 18]...
  36. Gu S, Chen K, Yin M, Wu Z, Wu Y. Proteomic profiling of isogenic primary and metastatic medulloblastoma cell lines reveals differential expression of key metastatic factors. J Proteomics. 2017;160:55-63 pubmed publisher
    ..primary and metastatic cell lines including known factors such as placental growth factor (PLGF), LIM homeobox 1 (LHX1) and prominim 1 (PROM1), as well as the negative regulator secreted protein acidic and cysteine rich (SPARC)...
  37. Wang Y, Stokes A, Duan Z, Hui J, Xu Y, Chen Y, et al. LDL Receptor-Related Protein 6 Modulates Ret Proto-Oncogene Signaling in Renal Development and Cystic Dysplasia. J Am Soc Nephrol. 2016;27:417-27 pubmed publisher
    ..The activities of other representative nephrogenic genes, including Lim1, Pax2, Pax8, GDNF, and Wnt11, were subsequently diminished in the mutant renal primordia...
  38. Weasner B, Kumar J. Competition among gene regulatory networks imposes order within the eye-antennal disc of Drosophila. Development. 2013;140:205-15 pubmed publisher
    ..the RD network leads to the inappropriate expression of several head capsule selector genes such as cut, Lim1 and wingless...
  39. Delbaere J, Vancamp P, Van Herck S, Bourgeois N, Green M, Wingate R, et al. MCT8 deficiency in Purkinje cells disrupts embryonic chicken cerebellar development. J Endocrinol. 2017;232:259-272 pubmed
    ..downregulation of the thyroid hormone-responsive gene ROR? and the Purkinje cell-specific differentiation marker LHX1/5 at day 6...
  40. Lim F, Ogawa S, Smith A, Parhar I. Proteomics Identification of Potential Candidates Involved in Cell Proliferation for Early Stage of Brain Regeneration in the Adult Zebrafish. Zebrafish. 2017;14:10-22 pubmed publisher
    ..Wnt inhibitory factor 1 [WIF1]), neuroprotection (metallothionein), cell proliferation (Spred2, ependymin, Lhx1, and Wnts), differentiation (Spred2, Lhx9, and Wnts), and morphogenesis (cytoplasmic actins and draculin)...
  41. Hadzic E, Catillon M, Halavatyi A, Medves S, Van Troys M, Moes M, et al. Delineating the Tes Interaction Site in Zyxin and Studying Cellular Effects of Its Disruption. PLoS ONE. 2015;10:e0140511 pubmed publisher
    ..Similar to zyxin, Tes harbors three highly conserved LIM domains of which the LIM1 domain directly interacts with zyxin...
  42. Chen C, Fu C, Lin Y, Chern S, Wu P, Chen Y, et al. Prenatal diagnosis of familial transmission of 17q12 microduplication associated with no apparent phenotypic abnormality. Taiwan J Obstet Gynecol. 2016;55:871-873 pubmed publisher
    ..arr 17q12 (34,822,465-36,243,365) × 3 encompassing 12 Online Mendelian Inheritance in Man (OMIM) genes including LHX1, ACACA, and HNF1B...
  43. González Peñas J, Amigo J, Santomé L, Sobrino B, Brenlla J, Agra S, et al. Targeted resequencing of regulatory regions at schizophrenia risk loci: Role of rare functional variants at chromatin repressive states. Schizophr Res. 2016;174:10-6 pubmed publisher
    ..34, 95% C.I.=2.45-2495.26). The variants are located at two genes affected by the 17q12 copy number variant, LHX1 and HNF1B...
  44. Sepponen K, Lundin K, Knuus K, Väyrynen P, Raivio T, Tapanainen J, et al. The Role of Sequential BMP Signaling in Directing Human Embryonic Stem Cells to Bipotential Gonadal Cells. J Clin Endocrinol Metab. 2017;102:4303-4314 pubmed publisher
    ..The two most efficient protocols consistently upregulated IM markers LHX1, PAX2, and OSR1 at days 2 to 4 and bipotential gonadal markers EMX2, GATA4, WT1, and LHX9 at day 8 of culture...
  45. Lui N, Tam W, Gao C, Huang J, Wang C, Jiang L, et al. Lhx1/5 control dendritogenesis and spine morphogenesis of Purkinje cells via regulation of Espin. Nat Commun. 2017;8:15079 pubmed publisher
    ..Here we report that specific inactivation of both Lhx1 and Lhx5 in postnatal PCs results in ataxic mutant mice with abnormal dendritic development...
  46. Joubert B, den Dekker H, Felix J, Bohlin J, Ligthart S, Beckett E, et al. Maternal plasma folate impacts differential DNA methylation in an epigenome-wide meta-analysis of newborns. Nat Commun. 2016;7:10577 pubmed publisher
    ..Most genes are not known for folate biology, including APC2, GRM8, SLC16A12, OPCML, PRPH, LHX1, KLK4 and PRSS21...
  47. Peng H, Talebzadeh Farrooji M, Osborne M, Prokop J, McDonald P, Karar J, et al. LIMD2 is a small LIM-only protein overexpressed in metastatic lesions that regulates cell motility and tumor progression by directly binding to and activating the integrin-linked kinase. Cancer Res. 2014;74:1390-1403 pubmed publisher
    ..was determined using nuclear magnetic resonance revealed a classic LIM-domain structure that was highly related to LIM1 of PINCH1, a core component of the integrin-linked kinase-parvin-pinch complex...
  48. Neuberg P, Hamaidi I, Danilin S, Ripoll M, Lindner V, Nothisen M, et al. Polydiacetylenic nanofibers as new siRNA vehicles for in vitro and in vivo delivery. Nanoscale. 2018;10:1587-1590 pubmed publisher
    ..Intraperitoneal injection of PDA-Nfs/siLim1 downregulated Lim-1 in subcutaneous tumor xenografts obtained with 786-O cells in nude mice. Thus, PDA-Nfs represent an innovative system for in vivo delivery of siRNAs. ..
  49. Sala S, Catillon M, Hadzic E, Schaffner Reckinger E, Van Troys M, Ampe C. The PET and LIM1-2 domains of testin contribute to intramolecular and homodimeric interactions. PLoS ONE. 2017;12:e0177879 pubmed publisher
    ..this extended model we determined that the testin region (amino acids 52-233) harbouring the PET domain interacts with the C-terminal LIM1-2 domains in vitro and in cells, and assign a critical role to tyrosine 288 in this interaction.
  50. Morona R, Ferran J, Puelles L, González A. Gene expression analysis of developing cell groups in the pretectal region of Xenopus laevis. J Comp Neurol. 2017;525:715-752 pubmed publisher
    ..Additional genoarchitectural information was provided by the expression of Gbx2, NPY, Lhx1, and Lhx9...
  51. Costello I, Nowotschin S, Sun X, Mould A, Hadjantonakis A, Bikoff E, et al. Lhx1 functions together with Otx2, Foxa2, and Ldb1 to govern anterior mesendoderm, node, and midline development. Genes Dev. 2015;29:2108-22 pubmed publisher
    ..Eomes, acting downstream from Nodal/Smad signals, directly activates the LIM domain homeobox transcription factor Lhx1 in the visceral endoderm...
  52. Teratani Ota Y, Yamamizu K, Piao Y, Sharova L, Amano M, Yu H, et al. Induction of specific neuron types by overexpression of single transcription factors. In Vitro Cell Dev Biol Anim. 2016;52:961-973 pubmed
    ..The majority of neuron-like cells generated by induction of Ascl1, Smad7, Nr2f1, Dlx2, Dlx4, Nr2f2, Barhl2, and Lhx1 were GABA-positive and expressed other markers of GABAergic neurons...
  53. Rasmussen M, Vestergaard E, Graakjaer J, Petkov Y, Bache I, Fagerberg C, et al. 17q12 deletion and duplication syndrome in Denmark-A clinical cohort of 38 patients and review of the literature. Am J Med Genet A. 2016;170:2934-2942 pubmed publisher
    ..The aberrations encompass the genes, HNF1B, LHX1, and ACACA, among others...
  54. Waschk D, Tewes A, Romer T, Hucke J, Kapczuk K, Schippert C, et al. Mutations in WNT9B are associated with Mayer-Rokitansky-Küster-Hauser syndrome. Clin Genet. 2016;89:590-6 pubmed publisher
    ..In previous studies, two of the patients with a WNT9B mutation were found to carry either an additional deletion of LHX1 or a missense mutation in TBX6...
  55. Cohen E, Maghnie M, Collot N, Leger J, Dastot F, Polak M, et al. Contribution of LHX4 Mutations to Pituitary Deficits in a Cohort of 417 Unrelated Patients. J Clin Endocrinol Metab. 2017;102:290-301 pubmed publisher
    ..As revealed by functional studies of LIM-defective recombinant LHX4 proteins, the LIM1 and LIM2 domains are not redundant...
  56. Stiegler A, Grant T, Luft J, Calderwood D, Snell E, Boggon T. Purification and SAXS analysis of the integrin linked kinase, PINCH, parvin (IPP) heterotrimeric complex. PLoS ONE. 2013;8:e55591 pubmed publisher
    ..we conduct a biochemical and structural analysis of the minimal IPP complex, comprising full-length human ILK, the LIM1 domain of PINCH1, and the CH2 domain of α-parvin...
  57. Ku H, Sun Y. Notch-dependent epithelial fold determines boundary formation between developmental fields in the Drosophila antenna. PLoS Genet. 2017;13:e1006898 pubmed publisher
    ..We studied the boundary separating the proximal A1 segment and the distal segments, defined respectively by Lim1 and Dll expression in the eye-antenna disc...
  58. Uluisik R, Romero E, Gadda G. Evidence for proton tunneling and a transient covalent flavin-substrate adduct in choline oxidase S101A. Biochim Biophys Acta Proteins Proteom. 2017;1865:1470-1478 pubmed publisher
    ..The limiting rate constants klim1 and klim2 at saturating substrate were well separated (klim1/klim2>9), ..
  59. Ledig S, Tewes A, Hucke J, Romer T, Kapczuk K, Schippert C, et al. Array-CGH Analysis in Patients with Müllerian Fusion Anomalies. Clin Genet. 2017;: pubmed publisher
    ..21, 9q33.1, 3q26.11 and 7q31.1. The rearrangement in 17q12 including LHX1 and HNF1? as well as in 22q11.21 have already been observed in MRKHS (Mayer-Rokitansky-Küster-Hauser syndrome)...
  60. Lim H, Hawkins J. Genetic control of gonadal differentiation. Baillieres Clin Endocrinol Metab. 1998;12:1-16 pubmed
    ..Several genes required for this complex developmental process have now been identified. The genes LIM1, WT1 and FTZ-F1 have been demonstrated to be involved in the formation of the gonads prior to their differentiation ..
  61. Reidy K, Rosenblum N. Cell and molecular biology of kidney development. Semin Nephrol. 2009;29:321-37 pubmed publisher
    ..Also emphasized are the roles of transcription factors Odd1, Eya1, Pax2, Lim1, and WT-1 in directing renal development...
  62. Dormoy V, Beraud C, Lindner V, Thomas L, Coquard C, Barthelmebs M, et al. LIM-class homeobox gene Lim1, a novel oncogene in human renal cell carcinoma. Oncogene. 2011;30:1753-63 pubmed publisher
    ..The transcription factor LIM-class homeobox gene Lim1 is required for normal organogenesis, including nephrogenesis, by regulating cell movements, differentiation and ..
  63. Dong W, Heng H, Lowsky R, Xu Y, DeCoteau J, Shi X, et al. Cloning, expression, and chromosomal localization to 11p12-13 of a human LIM/HOMEOBOX gene, hLim-1. DNA Cell Biol. 1997;16:671-8 pubmed
    ..Its expression in CML in blast crisis suggests that it may be involved with progression in this disease; a prospective study is required to confirm this. ..
  64. Guertl B, Senanayake U, Nusshold E, Leuschner I, Mannweiler S, Ebner B, et al. Lim1, an embryonal transcription factor, is absent in multicystic renal dysplasia, but reactivated in nephroblastomas. Pathobiology. 2011;78:210-9 pubmed publisher
    b>Lim1 (Lim homeobox 1) plays an important role during rodent renal development; however, its rolein human kidney development and disease is still unclear.
  65. Bozzi F, Bertuzzi S, Strina D, Giannetto C, Vezzoni P, Villa A. The exon-intron structure of human LHX1 gene. Biochem Biophys Res Commun. 1996;229:494-7 pubmed
    We have determined the genomic structure of the human LHX1 gene, a member of the LIM/homeobox (Lhx) gene family. The transcript is assembled from five exons, which are separated by introns ranging in size from 93 nt to 2.3 kb...
  66. Delay B, Corkins M, Hanania H, Salanga M, Deng J, Sudou N, et al. Tissue-Specific Gene Inactivation in Xenopus laevis: Knockout of lhx1 in the Kidney with CRISPR/Cas9. Genetics. 2018;208:673-686 pubmed publisher
    ..Our study shows that knockout of both homeologs of lhx1 results in the disruption of kidney development and function but does not lead to early developmental defects...
  67. Blixt M, Konjusha D, Ring H, Hallböök F. The zinc finger gene Nolz1 regulates the formation of retinal progenitor cells and suppresses the Lim3/Lhx3 phenotype of retinal bipolar cells in chicken retina. Dev Dyn. 2017;: pubmed publisher
    ..factor Nolz1 regulates spinal cord neuron development by interacting with the transcription factors Isl1, Lim1 and Lim3, which are also important for photoreceptors, horizontal and bipolar cells during retinal development...
  68. Shawlot W, Behringer R. Requirement for Lim1 in head-organizer function. Nature. 1995;374:425-30 pubmed
    b>Lim1 is a homeobox gene expressed in the organizer region of mouse embryos. To investigate the role of Lim1 during embryogenesis, a targeted deletion of the Lim1 gene was generated in embryonic stem cells...
  69. Brophy P, Rasmussen M, Parida M, Bonde G, Darbro B, Hong X, et al. A Gene Implicated in Activation of Retinoic Acid Receptor Targets Is a Novel Renal Agenesis Gene in Humans. Genetics. 2017;207:215-228 pubmed publisher
    ..Genetic investigations have identified several gene variants that cause RA, including EYA1, LHX1, and WT1 However, whereas compound null mutations of genes encoding ? and ? retinoic acid receptors (..
  70. Sandbacka M, Laivuori H, Freitas E, Halttunen M, Jokimaa V, Morin Papunen L, et al. TBX6, LHX1 and copy number variations in the complex genetics of Müllerian aplasia. Orphanet J Rare Dis. 2013;8:125 pubmed publisher
    ..We also sequenced LHX1 and found three apparently pathogenic missense variants in 5/112 patients...
  71. Atsuta Y, Takahashi Y. Early formation of the Müllerian duct is regulated by sequential actions of BMP/Pax2 and FGF/Lim1 signaling. Development. 2016;143:3549-3559 pubmed
    ..Subsequently, the BMP/Pax2 axis induces Lim1 expression, a hallmark of MD specification, for which FGF/ERK and WD-derived signals are also required...
  72. Ye L, Evans J, Gargett C. Lim1/LIM1 is expressed in developing and adult mouse and human endometrium. Histochem Cell Biol. 2012;137:527-36 pubmed publisher
    b>Lim1 encodes a homeodomain transcription factor required for head, kidney and female reproductive tract development in the murine embryo. Recently, Lim1 expression was documented in several adult murine and human organs...
  73. Xia M, Zhao H, Qin Y, Mu Y, Wang J, Bian Y, et al. LHX1 mutation screening in 96 patients with müllerian duct abnormalities. Fertil Steril. 2012;97:682-5 pubmed publisher
    To investigate whether LHX1 gene mutations exist in Han Chinese patients with müllerian duct abnormalities (MDAs). Mutation screening. University hospital. Ninety-six MDA patients and 105 control subjects from a Han Chinese population...
  74. Williams L, Demir Eksi D, Shen Y, Lossie A, Chorich L, Sullivan M, et al. Genetic analysis of Mayer-Rokitansky-Kuster-Hauser syndrome in a large cohort of families. Fertil Steril. 2017;108:145-151.e2 pubmed publisher
    ..Some of the top candidate genes are WNT4, HNF1B, and LHX1. The goals of this study were to: 1) determine the prevalence of WNT4, HNF1B, and LHX1 point mutations, as well as ..
  75. Ou Y, Wu Q, Wu C, Liu X, Song Y, Zhan Q. Migfilin promotes migration and invasion in glioma by driving EGFR and MMP-2 signalings: A positive feedback loop regulation. J Genet Genomics. 2017;44:557-565 pubmed publisher
    ..Functional and structural studies showed that the LIM1 domain of Migfilin was required for Migfilin-mediated TIMP2 expression inhibition and MMP-2 activity, and was also ..
  76. Mumert M, Dubuc A, Wu X, Northcott P, Chin S, Pedone C, et al. Functional genomics identifies drivers of medulloblastoma dissemination. Cancer Res. 2012;72:4944-53 pubmed publisher
    ..We show here that ectopic expression of Eras, Lhx1, Ccrk, and Akt shifted the in vivo growth characteristics of Shh-induced medulloblastomas from a localized pattern ..
  77. Christopoulos P, Gazouli M, Fotopoulou G, Creatsas G. The role of genes in the development of Mullerian anomalies: where are we today?. Obstet Gynecol Surv. 2009;64:760-8 pubmed publisher
  78. Simon C, Downes D, Gosden M, Telenius J, Higgs D, Hughes J, et al. Functional characterisation of cis-regulatory elements governing dynamic Eomes expression in the early mouse embryo. Development. 2017;144:1249-1260 pubmed publisher
    ..By contrast, activation of the Eomes target genes Foxa2 and Lhx1 is associated with higher order chromatin reorganisation...
  79. Foucher I, Montesinos M, Volovitch M, Prochiantz A, Trembleau A. Joint regulation of the MAP1B promoter by HNF3beta/Foxa2 and Engrailed is the result of a highly conserved mechanism for direct interaction of homeoproteins and Fox transcription factors. Development. 2003;130:1867-76 pubmed
    ..Finally, Foxa2 not only binds Engrailed but also Lim1, Gsc and Hoxa5 homeoproteins and in the four cases Foxa2 binds at least the homeodomain...
  80. Phillips J. Assignment of LHX1 to human chromosome bands 17q11.2-->q12 by use of radiation hybrid mapping and somatic cell hybridization. Cytogenet Genome Res. 2002;97:140D pubmed
  81. Ostendorff H, Peirano R, Peters M, Schlüter A, Bossenz M, Scheffner M, et al. Ubiquitination-dependent cofactor exchange on LIM homeodomain transcription factors. Nature. 2002;416:99-103 pubmed
    ..Our data provide a mechanistic basis for cofactor exchange on DNA-bound transcription factors, and probably represent a general mechanism of transcriptional regulation. ..
  82. Nakano T, Murata T, Matsuo I, Aizawa S. OTX2 directly interacts with LIM1 and HNF-3beta. Biochem Biophys Res Commun. 2000;267:64-70 pubmed
    ..in these tissues, and mutant mice analyses have suggested the interactions of the Otx2 gene cascade with the Lim1 or HNF-3beta cascade...
  83. Zhang W, Zhou X, Liu L, Zhu Y, Liu C, Pan H, et al. Identification and functional analysis of a novel LHX1 mutation associated with congenital absence of the uterus and vagina. Oncotarget. 2017;8:8785-8790 pubmed publisher
    ..Here, utilizing whole-exome sequencing (WES), we identified one novel missense mutation in LHX1 (NM_005568: c.G1108A, p.A370T) in one of ten unrelated patients diagnosed with CAUV...
  84. Jurata L, Pfaff S, Gill G. The nuclear LIM domain interactor NLI mediates homo- and heterodimerization of LIM domain transcription factors. J Biol Chem. 1998;273:3152-7 pubmed
    ..The combinatorial expression of the LIM homeodomain proteins Isl1, Isl2, Lhx1, and Lhx3 in subsets of developing motor neurons correlates with the future organization of these neurons into ..
  85. Chung E, Deacon P, Park J. Notch is required for the formation of all nephron segments and primes nephron progenitors for differentiation. Development. 2017;144:4530-4539 pubmed publisher
    ..Cells lacking Notch fail to form the S-shaped body and show reduced expression of Lhx1 and Hnf1b Consistent with this, we find that constitutive activation of Notch in mesenchymal nephron ..