Gene Symbol: Osr1
Description: odd-skipped related transcription factor 1
Alias: Odd1, Osr, protein odd-skipped-related 1, odd-skipped related 1
Species: mouse
Products:     Osr1

Top Publications

  1. Mugford J, Sipila P, McMahon J, McMahon A. Osr1 expression demarcates a multi-potent population of intermediate mesoderm that undergoes progressive restriction to an Osr1-dependent nephron progenitor compartment within the mammalian kidney. Dev Biol. 2008;324:88-98 pubmed publisher
    ..molecular fate mapping to demonstrate that the majority of cell types within the metanephric kidney arise from an Osr1(+) population of metanephric progenitor cells...
  2. James R, Kamei C, Wang Q, Jiang R, Schultheiss T. Odd-skipped related 1 is required for development of the metanephric kidney and regulates formation and differentiation of kidney precursor cells. Development. 2006;133:2995-3004 pubmed
    ..Here we report that the gene odd-skipped related 1 (Odd1) plays an important role in both these processes...
  3. So P, Danielian P. Cloning and expression analysis of a mouse gene related to Drosophila odd-skipped. Mech Dev. 1999;84:157-60 pubmed
    ..Here we report the cloning and expression analysis of a mouse gene related to odd, odd-skipped related 1 (Osr1)...
  4. Hu Y, Okumura L, Page D. Gata4 is required for formation of the genital ridge in mice. PLoS Genet. 2013;9:e1003629 pubmed publisher
    ..Our data indicate that GATA4 is required to initiate formation of the genital ridge in both XX and XY fetuses, prior to its previously reported role in testicular differentiation of the XY gonad. ..
  5. Wang Q, Lan Y, Cho E, Maltby K, Jiang R. Odd-skipped related 1 (Odd 1) is an essential regulator of heart and urogenital development. Dev Biol. 2005;288:582-94 pubmed
    ..These data provide new insights into the molecular mechanisms underlying heart morphogenesis and urogenital development. ..
  6. Debeer P, de Ravel T, Devriendt K, Fryns J, Huysmans C, Van de Ven W. Human homologues of Osr1 and Osr2 are not involved in a syndrome with distal limb deficiencies, oral abnormalities, and renal defects. Am J Med Genet. 2002;111:455-6 pubmed
  7. Bohnenpoll T, Bettenhausen E, Weiss A, Foik A, Trowe M, Blank P, et al. Tbx18 expression demarcates multipotent precursor populations in the developing urogenital system but is exclusively required within the ureteric mesenchymal lineage to suppress a renal stromal fate. Dev Biol. 2013;380:25-36 pubmed publisher
    ..Our study provides new insights into the molecular diversity of urogenital progenitor cells and helps to understand the specification of the ureteric mesenchymal sub-lineage...
  8. Stricker S, Brieske N, Haupt J, Mundlos S. Comparative expression pattern of Odd-skipped related genes Osr1 and Osr2 in chick embryonic development. Gene Expr Patterns. 2006;6:826-34 pubmed
    ..In mammals two paralogs exist, Osr1 and Osr2, with functions described in heart and urogenital, and in secondary palate development, respectively...
  9. Lan Y, Liu H, Ovitt C, Jiang R. Generation of Osr1 conditional mutant mice. Genesis. 2011;49:419-22 pubmed publisher
    The Odd-skipped related 1 (Osr1) gene encodes a zinc finger protein homologous to the Drosophila Odd-skipped transcription factor...

More Information


  1. Lan Y, Kingsley P, Cho E, Jiang R. Osr2, a new mouse gene related to Drosophila odd-skipped, exhibits dynamic expression patterns during craniofacial, limb, and kidney development. Mech Dev. 2001;107:175-9 pubmed
    ..identity overall and 98% sequence identity in the zinc finger region, respectively, with the previously reported Osr1 gene product. During mouse embryonic development, Osr2 expression is first detected at E9...
  2. Taguchi A, Kaku Y, Ohmori T, Sharmin S, Ogawa M, Sasaki H, et al. Redefining the in vivo origin of metanephric nephron progenitors enables generation of complex kidney structures from pluripotent stem cells. Cell Stem Cell. 2014;14:53-67 pubmed publisher
    ..Unexpectedly, we find that posteriorly located T(+) MM precursors are developmentally distinct from Osr1(+) ureteric bud progenitors during the postgastrulation stage, and we identify phasic Wnt stimulation and stage-..
  3. Hu Y, Nicholls P, Soh Y, Daniele J, Junker J, van Oudenaarden A, et al. Licensing of primordial germ cells for gametogenesis depends on genital ridge signaling. PLoS Genet. 2015;11:e1005019 pubmed publisher
    ..However, these germ cells did not undergo licensing and instead retained characteristics of PGCs. Our results indicate that licensing is not purely cell-autonomous but is induced by the somatic genital ridge. ..
  4. McJunkin K, Mazurek A, Premsrirut P, Zuber J, Dow L, Simon J, et al. Reversible suppression of an essential gene in adult mice using transgenic RNA interference. Proc Natl Acad Sci U S A. 2011;108:7113-8 pubmed publisher
    ..Overall, we have established a robust system that serves the purpose of temperature-sensitive alleles in other model organisms, enabling inducible and reversible suppression of essential genes in a mammalian system. ..
  5. Gao Y, Lan Y, Ovitt C, Jiang R. Functional equivalence of the zinc finger transcription factors Osr1 and Osr2 in mouse development. Dev Biol. 2009;328:200-9 pubmed publisher
    b>Osr1 and Osr2 are the only mammalian homologs of the Drosophila odd-skipped family developmental regulators...
  6. Wilm B, James R, Schultheiss T, Hogan B. The forkhead genes, Foxc1 and Foxc2, regulate paraxial versus intermediate mesoderm cell fate. Dev Biol. 2004;271:176-89 pubmed
    ..Taken together, the data indicate that Foxc1 and Foxc2 regulate the establishment of paraxial versus intermediate mesoderm cell fates in the vertebrate embryo. ..
  7. Xu J, Xu P. Eya-six are necessary for survival of nephrogenic cord progenitors and inducing nephric duct development before ureteric bud formation. Dev Dyn. 2015;244:866-73 pubmed publisher
    ..Our data support a model where Eya-Six may form a complex to regulate nephron progenitor cell development before metanephric specification and are critical mesenchymal factors for inducing nephric duct development. ..
  8. Yokoyama S, Furukawa S, Kitada S, Mori M, Saito T, Kawakami K, et al. Analysis of transcription factors expressed at the anterior mouse limb bud. PLoS ONE. 2017;12:e0175673 pubmed publisher
    ..Our results demonstrate that gene expression analysis based on EMBRYS could contribute to the identification of genes that play a role in patterning of the limb mesenchyme. ..
  9. Yamauchi M, Kawai S, Kato T, Ooshima T, Amano A. Odd-skipped related 1 gene expression is regulated by Runx2 and Ikzf1 transcription factors. Gene. 2008;426:81-90 pubmed publisher
    Odd-skipped related 1 (Osr1) gene encodes a zinc-finger transcription factor that plays important roles in embryonic, heart, and urogenital development, however, it is unknown how its expression is regulated...
  10. Wainwright E, Wilhelm D, Combes A, Little M, Koopman P. ROBO2 restricts the nephrogenic field and regulates Wolffian duct-nephrogenic cord separation. Dev Biol. 2015;404:88-102 pubmed publisher
    ..These insights illuminate the aetiology of multiplex kidney formation in human individuals with ROBO2 mutations. ..
  11. Norden J, Grieskamp T, Lausch E, van Wijk B, van den Hoff M, Englert C, et al. Wt1 and retinoic acid signaling in the subcoelomic mesenchyme control the development of the pleuropericardial membranes and the sinus horns. Circ Res. 2010;106:1212-20 pubmed publisher
    ..Thus, our results provide novel insight into the genetic and cellular pathways regulating the posterior extension of the mammalian heart and the formation of its coelomic lining. ..
  12. Kawai S, Yamauchi M, Wakisaka S, Ooshima T, Amano A. Zinc-finger transcription factor odd-skipped related 2 is one of the regulators in osteoblast proliferation and bone formation. J Bone Miner Res. 2007;22:1362-72 pubmed
    ..In this study, N-terminal-deleted Osr2 was found to act as a dominant-negative mutant toward both Osr1 and Osr2...
  13. Chiga M, Rafiqi F, Alessi D, Sohara E, Ohta A, Rai T, et al. Phenotypes of pseudohypoaldosteronism type II caused by the WNK4 D561A missense mutation are dependent on the WNK-OSR1/SPAK kinase cascade. J Cell Sci. 2011;124:1391-5 pubmed publisher
    ..Although previous in vitro studies had suggested the existence of a phosphorylation cascade involving the WNK, OSR1 and SPAK kinases, whether the WNK-OSR1/SPAK cascade is in fact fully responsible for NCC phosphorylation in vivo ..
  14. Yumoto K, Thomas P, Lane J, Matsuzaki K, Inagaki M, Ninomiya Tsuji J, et al. TGF-?-activated kinase 1 (Tak1) mediates agonist-induced Smad activation and linker region phosphorylation in embryonic craniofacial neural crest-derived cells. J Biol Chem. 2013;288:13467-80 pubmed publisher
    ..The role of Smad-independent TGF-? signaling in craniofacial development is poorly elucidated...
  15. Mae S, Shirasawa S, Yoshie S, Sato F, Kanoh Y, Ichikawa H, et al. Combination of small molecules enhances differentiation of mouse embryonic stem cells into intermediate mesoderm through BMP7-positive cells. Biochem Biophys Res Commun. 2010;393:877-82 pubmed publisher
    ..When these cells were cultured with adding retinoic acid, expression of odd-skipped related 1 (Osr1), which is essential to IM differentiation, was enhanced...
  16. Zhou L, Liu J, Olson P, Zhang K, Wynne J, Xie L. Tbx5 and Osr1 interact to regulate posterior second heart field cell cycle progression for cardiac septation. J Mol Cell Cardiol. 2015;85:1-12 pubmed publisher
    ..Previous studies have demonstrated that Tbx5 regulates Osr1 expression in the second heart field (SHF) of E9.5 mouse embryos...
  17. Mugford J, Sipila P, Kobayashi A, Behringer R, McMahon A. Hoxd11 specifies a program of metanephric kidney development within the intermediate mesoderm of the mouse embryo. Dev Biol. 2008;319:396-405 pubmed publisher
    ..This program maintains tubule forming progenitors and instructs a metanephric specific pattern of nephron differentiation. ..
  18. Ferdaus M, Barber K, López Cayuqueo K, Terker A, Argaiz E, Gassaway B, et al. SPAK and OSR1 play essential roles in potassium homeostasis through actions on the distal convoluted tubule. J Physiol. 2016;594:4945-66 pubmed publisher
    STE20 (Sterile 20)/SPS-1 related proline/alanine-rich kinase (SPAK) and oxidative stress-response kinase-1 (OSR1) phosphorylate and activate the renal Na(+) -K(+) -2Cl(-) cotransporter 2 (NKCC2) and Na(+) Cl(-) cotransporter (NCC)...
  19. Zhang K, Xiang M, Zhou L, Liu J, Curry N, Heine Suñer D, et al. Gene network and familial analyses uncover a gene network involving Tbx5/Osr1/Pcsk6 interaction in the second heart field for atrial septation. Hum Mol Genet. 2016;25:1140-51 pubmed publisher
    ..Our previous studies have demonstrated a genetic interaction between Tbx5 and Osr1 in the second heart field (SHF) for atrial septation...
  20. Dbouk H, Weil L, Perera G, Dellinger M, Pearson G, Brekken R, et al. Actions of the protein kinase WNK1 on endothelial cells are differentially mediated by its substrate kinases OSR1 and SPAK. Proc Natl Acad Sci U S A. 2014;111:15999-6004 pubmed publisher
    ..be rescued by endothelial-specific expression of a constitutively active form of the WNK1 substrate protein kinase OSR1 (oxidative stress responsive 1)...
  21. Geng Y, Hoke A, Delpire E. The Ste20 kinases Ste20-related proline-alanine-rich kinase and oxidative-stress response 1 regulate NKCC1 function in sensory neurons. J Biol Chem. 2009;284:14020-8 pubmed publisher
    ..bind and phosphorylate NKCC1: Ste20-related proline-alanine-rich kinase (SPAK) and oxidative-stress response 1 (OSR1)...
  22. Di Giovanni V, Walker K, Bushnell D, Schaefer C, Sims Lucas S, Puri P, et al. Fibroblast growth factor receptor-Frs2α signaling is critical for nephron progenitors. Dev Biol. 2015;400:82-93 pubmed publisher
    ..Thus, Fgfr1 and Fgfr2 have synergistic roles in maintaining nephron progenitors; furthermore, Fgfr signaling in nephron progenitors appears to be mediated predominantly by Frs2α. ..
  23. Xu J, Liu H, Chai O, Lan Y, Jiang R. Osr1 Interacts Synergistically with Wt1 to Regulate Kidney Organogenesis. PLoS ONE. 2016;11:e0159597 pubmed publisher
    ..Recent studies have associated a variant of the OSR1 gene with reduction of newborn kidney size and function in heterozygotes and neonatal lethality with kidney defects ..
  24. Han L, Xu J, Grigg E, Slack M, Chaturvedi P, Jiang R, et al. Osr1 functions downstream of Hedgehog pathway to regulate foregut development. Dev Biol. 2017;427:72-83 pubmed publisher
    ..We show that the zinc finger transcription factor Osr1 is a novel HH target as Osr1 expression in the foregut mesenchyme depends on HH signaling and the effector of HH ..
  25. Pathare G, Foller M, Daryadel A, Mutig K, Bogatikov E, Fajol A, et al. OSR1-sensitive renal tubular phosphate reabsorption. Kidney Blood Press Res. 2012;36:149-61 pubmed publisher
    The oxidative stress-responsive kinase 1 (OSR1) participates in the WNK-(with no K) kinase dependent regulation of renal salt excretion and blood pressure...
  26. Gerber S, Steinberg F, Beyeler M, Villiger P, Trueb B. The murine Fgfrl1 receptor is essential for the development of the metanephric kidney. Dev Biol. 2009;335:106-19 pubmed publisher
    ..We also observed a loss of Pax2 positive nephron precursor cells and an increase of apoptosis in the cortical zone of the remnant kidney. Fgfrl1 is therefore essential for mesenchymal differentiation in the early steps of nephrogenesis. ..
  27. Cain J, Islam E, Haxho F, Chen L, Bridgewater D, Nieuwenhuis E, et al. GLI3 repressor controls nephron number via regulation of Wnt11 and Ret in ureteric tip cells. PLoS ONE. 2009;4:e7313 pubmed publisher
    ..Thus, GLI3 repressor controls nephron number by regulating ureteric tip cell expression of Wnt11 and Ret. ..
  28. Pauws E, Hoshino A, Bentley L, Prajapati S, Keller C, Hammond P, et al. Tbx22null mice have a submucous cleft palate due to reduced palatal bone formation and also display ankyloglossia and choanal atresia phenotypes. Hum Mol Genet. 2009;18:4171-9 pubmed publisher
    ..These findings could have important implications for the molecular diagnosis in patients with isolated SMCP and/or unexplained choanal atresia...
  29. Zhang Z, Iglesias D, Eliopoulos N, El Kares R, Chu L, Romagnani P, et al. A variant OSR1 allele which disturbs OSR1 mRNA expression in renal progenitor cells is associated with reduction of newborn kidney size and function. Hum Mol Genet. 2011;20:4167-74 pubmed publisher
    ..Here, we hypothesize that dysfunctional variants of the Odd-Skipped Related 1 (OSR1) gene which compromise the renal progenitor cell pool might also limit newborn kidney size and function...
  30. Jeong J, Cesario J, Zhao Y, Burns L, Westphal H, Rubenstein J. Cleft palate defect of Dlx1/2-/- mutant mice is caused by lack of vertical outgrowth in the posterior palate. Dev Dyn. 2012;241:1757-69 pubmed publisher
    ..Therefore, we analyzed the morphological, cellular, and molecular changes caused by inactivation of Dlx1 and Dlx2 as related to palate development...
  31. Kobayashi H, Kawakami K, Asashima M, Nishinakamura R. Six1 and Six4 are essential for Gdnf expression in the metanephric mesenchyme and ureteric bud formation, while Six1 deficiency alone causes mesonephric-tubule defects. Mech Dev. 2007;124:290-303 pubmed
    ..These results highlight the fact that Six1 and Six4 have collaborative functions in the metanephros but not in the mesonephros. ..
  32. Vorontsova I, Lam L, Delpire E, Lim J, Donaldson P. Identification of the WNK-SPAK/OSR1 signaling pathway in rodent and human lenses. Invest Ophthalmol Vis Sci. 2014;56:310-21 pubmed publisher
    ..The expression of with-no-lysine kinase (WNK 1, 3, 4), oxidative stress response kinase 1 (OSR1), and Ste20-like proline alanine rich kinase (SPAK) were determined at either the transcript or protein levels in ..
  33. Elvira B, Munoz C, Borras J, Chen H, Warsi J, Ajay S, et al. SPAK and OSR1 dependent down-regulation of murine renal outer medullary K channel ROMK1. Kidney Blood Press Res. 2014;39:353-60 pubmed publisher
    The kinases SPAK (SPS1-related proline/alanine-rich kinase) and OSR1 (oxidative stress-responsive kinase 1) participate in the regulation of the NaCl cotransporter NCC and the Na+, K+, 2Cl- cotransporter NKCC2...
  34. Rana M, Théveniau Ruissy M, De Bono C, Mesbah K, Francou A, Rammah M, et al. Tbx1 coordinates addition of posterior second heart field progenitor cells to the arterial and venous poles of the heart. Circ Res. 2014;115:790-9 pubmed publisher
    ..Our results provide new insights into the pathogenesis of congenital heart defects and 22q11.2 deletion syndrome phenotypes. ..
  35. Xu J, Liu H, Park J, Lan Y, Jiang R. Osr1 acts downstream of and interacts synergistically with Six2 to maintain nephron progenitor cells during kidney organogenesis. Development. 2014;141:1442-52 pubmed publisher
    ..We report here that Six2 is required to maintain expression of Osr1, a homolog of the Drosophila odd-skipped zinc-finger transcription factor, in the undifferentiated cap mesenchyme...
  36. Lan Y, Ovitt C, Cho E, Maltby K, Wang Q, Jiang R. Odd-skipped related 2 (Osr2) encodes a key intrinsic regulator of secondary palate growth and morphogenesis. Development. 2004;131:3207-16 pubmed
    ..The developmental timing of palatal growth retardation correlates exactly with the spatiotemporal pattern of Osr1 gene expression during palate development...
  37. Xie J, Yoon J, Yang S, Lin S, Huang C. WNK1 protein kinase regulates embryonic cardiovascular development through the OSR1 signaling cascade. J Biol Chem. 2013;288:8566-74 pubmed publisher
    ..We found that mice with a global deletion of Osr1, which encodes oxidative stress-responsive kinase-1, a protein kinase activated by WNK1, died in utero beginning at ..
  38. Murray S, Oram K, Gridley T. Multiple functions of Snail family genes during palate development in mice. Development. 2007;134:1789-97 pubmed
  39. Richardson C, Sakamoto K, de los Heros P, Deak M, Campbell D, Prescott A, et al. Regulation of the NKCC2 ion cotransporter by SPAK-OSR1-dependent and -independent pathways. J Cell Sci. 2011;124:789-800 pubmed publisher
    ..We demonstrate that hypotonic low-chloride conditions that activate the WNK1-SPAK and OSR1 pathway promote phosphorylation of NKCC2 isoforms (A, B and F) at five residues (Ser91, Thr95, Thr100, Thr105 and ..
  40. Oeda S, Hayashi Y, Chan T, Takasato M, Aihara Y, Okabayashi K, et al. Induction of intermediate mesoderm by retinoic acid receptor signaling from differentiating mouse embryonic stem cells. Int J Dev Biol. 2013;57:383-9 pubmed publisher
    ..mESC cultures induced the expression of marker genes and proteins for intermediate mesoderm, odd-skipped related 1 (Osr1) and Wilm?s Tumor 1 (Wt1)...
  41. Li Y, Liu J, McLaughlin N, Bachvarov D, Saifudeen Z, El Dahr S. Genome-wide analysis of the p53 gene regulatory network in the developing mouse kidney. Physiol Genomics. 2013;45:948-64 pubmed publisher
    ..We conclude p53 targets transcriptional networks regulating nephrogenesis and cellular metabolism during kidney development. ..
  42. Wang J, Bai Y, Li H, Greene S, Klysik E, Yu W, et al. MicroRNA-17-92, a direct Ap-2? transcriptional target, modulates T-box factor activity in orofacial clefting. PLoS Genet. 2013;9:e1003785 pubmed publisher
    ..Our data are the first genetic evidence that an individual miR or miR cluster is functionally important in mammalian CL/P. ..
  43. Blake J, Hu D, Cain J, Rosenblum N. Urogenital development in Pallister-Hall syndrome is disrupted in a cell-lineage-specific manner by constitutive expression of GLI3 repressor. Hum Mol Genet. 2016;25:437-47 pubmed publisher
    ..We conclude that the urogenital system phenotypes observed in PHS are caused by GLI3R-dependent perturbations in nephric duct patterning, renal branching morphogenesis and nephrogenic progenitor self-renewal. ..
  44. Gao Y, Lan Y, Liu H, Jiang R. The zinc finger transcription factors Osr1 and Osr2 control synovial joint formation. Dev Biol. 2011;352:83-91 pubmed publisher
    ..Here we report that Osr1 and Osr2, the mammalian homologs of the odd-skipped family of zinc finger transcription factors that are required ..
  45. Liu H, Lan Y, Xu J, Chang C, Brugmann S, Jiang R. Odd-skipped related-1 controls neural crest chondrogenesis during tongue development. Proc Natl Acad Sci U S A. 2013;110:18555-60 pubmed publisher
    ..We show here that the odd-skipped related-1 (Osr1) transcription factor is expressed throughout the neural crest-derived tongue mesenchyme in mouse, but not in chick,..
  46. Davisson M, Cook S, Akeson E, Liu D, Heffner C, Gudis P, et al. Kidney adysplasia and variable hydronephrosis, a new mutation affecting the odd-skipped related 1 gene in the mouse, causes variable defects in kidney development and hydronephrosis. Am J Physiol Renal Physiol. 2015;308:F1335-42 pubmed publisher
    Many genes, including odd-skipped related 1 (Osr1), are involved in regulation of mammalian kidney development...
  47. Ranghini E, Dressler G. Evidence for intermediate mesoderm and kidney progenitor cell specification by Pax2 and PTIP dependent mechanisms. Dev Biol. 2015;399:296-305 pubmed publisher
    ..These data provide evidence that Pax2 specifies the intermediate mesoderm and renal epithelial cells through epigenetic mechanisms and in part by repressing paraxial mesodermal fate. ..
  48. Fillion M, El Andalousi J, Tokhmafshan F, Murugapoopathy V, Watt C, Murawski I, et al. Heterozygous loss-of-function mutation in Odd-skipped related 1 (Osr1) is associated with vesicoureteric reflux, duplex systems, and hydronephrosis. Am J Physiol Renal Physiol. 2017;313:F1106-F1115 pubmed publisher
    Odd-skipped related 1 (Osr1) is a transcriptional repressor that plays critical roles in maintaining the mesenchymal stem cell population within the developing kidney...