|
|
|
|
|
|
|
||||
| Home Help Feedback Subscriptions Archive Search Table of Contents | ||||
Development, Vol 120, Issue 7 2065-2075, Copyright © 1994 by Company of Biologists
JOURNAL ARTICLES |
W Wurst, AB Auerbach and AL Joyner
Division of Molecular and Developmental Biology, Samuel Lunenfeld Research Institute, Mt. Sinai Hospital, Toronto, Canada.
During mouse development, the homeobox-containing gene En-1 is specifically expressed across the mid-hindbrain junction, the ventral ectoderm of the limb buds, and in regions of the hindbrain, spinal cord, somites and somite-derived tissues. To address the function of En-1 during embryogenesis, we have generated mice homozygous for a targeted deletion of the En-1 homeobox. En-1 mutant mice died shortly after birth and exhibited multiple developmental defects. In the brains of newborn mutants, most of the colliculi and cerebellum were missing and the third and fourth cranial nerves were absent. A deletion of midhindbrain tissue was observed as early as 9.5 days of embryonic development and the phenotype resembles that previously reported for Wnt-1 mutant mice. In addition, patterning of the forelimb paws and sternum was disrupted, and the 13th ribs were truncated. The results of these studies suggest a cell autonomous role for En-1 in generation and/or survival of mid-hindbrain precursor cells and also a non-cell autonomous role in signalling normal development of the limbs and possibly sternum.
This article has been cited by other articles:
|
|
 |
|
  E. Hedlund, J. Pruszak, T. Lardaro, W. Ludwig, A. Vinuela, K.-S. Kim, and O. Isacson Embryonic Stem Cell-Derived Pitx3-Enhanced Green Fluorescent Protein Midbrain Dopamine Neurons Survive Enrichment by Fluorescence-Activated Cell Sorting and Function in an Animal Model of Parkinson's Disease Stem Cells, June 1, 2008; 26(6): 1526 - 1536. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. A. Gray Transcription factors and the genetic organization of brain stem respiratory neurons J Appl Physiol, May 1, 2008; 104(5): 1513 - 1521. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Yang, Z.-J. Zhang, M. Oldenburg, M. Ayala, and S.-C. Zhang Human Embryonic Stem Cell-Derived Dopaminergic Neurons Reverse Functional Deficit in Parkinsonian Rats Stem Cells, January 1, 2008; 26(1): 55 - 63. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. K. Sgaier, Z. Lao, M. P. Villanueva, F. Berenshteyn, D. Stephen, R. K. Turnbull, and A. L. Joyner Genetic subdivision of the tectum and cerebellum into functionally related regions based on differential sensitivity to engrailed proteins Development, June 15, 2007; 134(12): 2325 - 2335. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. L. Andoniadou, M. Signore, E. Sajedi, C. Gaston-Massuet, D. Kelberman, A. J. Burns, N. Itasaki, M. Dattani, and J. P. Martinez-Barbera Lack of the murine homeobox gene Hesx1 leads to a posterior transformation of the anterior forebrain Development, April 15, 2007; 134(8): 1499 - 1508. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Sonnier, G. Le Pen, A. Hartmann, J.-C. Bizot, F. Trovero, M.-O. Krebs, and A. Prochiantz Progressive Loss of Dopaminergic Neurons in the Ventral Midbrain of Adult Mice Heterozygote for Engrailed1 J. Neurosci., January 31, 2007; 27(5): 1063 - 1071. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Guo, H.-Y. Qiu, Y. Huang, H. Chen, R.-Q. Yang, S.-D. Chen, R. L. Johnson, Z.-F. Chen, and Y.-Q. Ding Lmx1b is essential for Fgf8 and Wnt1 expression in the isthmic organizer during tectum and cerebellum development in mice Development, January 15, 2007; 134(2): 317 - 325. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. Foucher, M. Mione, A. Simeone, D. Acampora, L. Bally-Cuif, and C. Houart Differentiation of cerebellar cell identities in absence of Fgf signalling in zebrafish Otx morphants Development, May 15, 2006; 133(10): 1891 - 1900. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Griesel, D. Treichel, P. Collombat, J. Krull, A. Zembrzycki, W. M. R. van den Akker, P. Gruss, A. Simeone, and A. Mansouri Sp8 controls the anteroposterior patterning at the midbrain-hindbrain border Development, May 1, 2006; 133(9): 1779 - 1787. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Kele, N. Simplicio, A. L. M. Ferri, H. Mira, F. Guillemot, E. Arenas, and S.-L. Ang Neurogenin 2 is required for the development of ventral midbrain dopaminergic neurons Development, February 1, 2006; 133(3): 495 - 505. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. A. Deckelbaum, A. Majithia, T. Booker, J. E. Henderson, and C. A. Loomis The homeoprotein engrailed 1 has pleiotropic functions in calvarial intramembranous bone formation and remodeling Development, January 1, 2006; 133(1): 63 - 74. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. M. Louie and J. G. Gleeson Genetic basis of Joubert syndrome and related disorders of cerebellar development Hum. Mol. Genet., October 15, 2005; 14(suppl_2): R235 - R242. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. P. O'Hara, E. Beck, L. K. Barr, L. L. Wong, D. S. Kessler, and R. D. Riddle Zebrafish Lmx1b.1 and Lmx1b.2 are required for maintenance of the isthmic organizer Development, July 15, 2005; 132(14): 3163 - 3173. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Vernay, M. Koch, F. Vaccarino, J. Briscoe, A. Simeone, R. Kageyama, and S.-L. Ang Otx2 Regulates Subtype Specification and Neurogenesis in the Midbrain J. Neurosci., May 11, 2005; 25(19): 4856 - 4867. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. S. Banting, O. Barak, T. M. Ames, A. C. Burnham, M. D. Kardel, N. S. Cooch, C. E. Davidson, R. Godbout, H. E. McDermid, and R. Shiekhattar CECR2, a protein involved in neurulation, forms a novel chromatin remodeling complex with SNF2L Hum. Mol. Genet., February 15, 2005; 14(4): 513 - 524. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Ohtoshi and R. R. Behringer Neonatal Lethality, Dwarfism, and Abnormal Brain Development in Dmbx1 Mutant Mice Mol. Cell. Biol., September 1, 2004; 24(17): 7548 - 7558. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Sock, S. D. Rettig, J. Enderich, M. R. Bosl, E. R. Tamm, and M. Wegner Gene Targeting Reveals a Widespread Role for the High-Mobility-Group Transcription Factor Sox11 in Tissue Remodeling Mol. Cell. Biol., August 1, 2004; 24(15): 6635 - 6644. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. P. Smidt, S. M. Smits, H. Bouwmeester, F. P. T. Hamers, A. J. A. van der Linden, A. J. C. G. M. Hellemons, J. Graw, and J. P. H. Burbach Early developmental failure of substantia nigra dopamine neurons in mice lacking the homeodomain gene Pitx3 Development, March 1, 2004; 131(5): 1145 - 1155. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Louvi, P. Alexandre, C. Metin, W. Wurst, and M. Wassef The isthmic neuroepithelium is essential for cerebellar midline fusion Development, November 15, 2003; 130(22): 5319 - 5330. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Scholpp, C. Lohs, and M. Brand Engrailed and Fgf8 act synergistically to maintain the boundary between diencephalon and mesencephalon Development, October 15, 2003; 130(20): 4881 - 4893. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Marie and J. M. Blagburn Differential Roles of Engrailed Paralogs in Determining Sensory Axon Guidance and Synaptic Target Recognition J. Neurosci., August 27, 2003; 23(21): 7854 - 7862. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Holzschuh, G. Hauptmann, and W. Driever Genetic Analysis of the Roles of Hh, FGF8, and Nodal Signaling during Catecholaminergic System Development in the Zebrafish Brain J. Neurosci., July 2, 2003; 23(13): 5507 - 5519. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. A. Bilovocky, R. R. Romito-DiGiacomo, C. L. Murcia, S. M. Maricich, and K. Herrup Factors in the Genetic Background Suppress the Engrailed-1 Cerebellar Phenotype J. Neurosci., June 15, 2003; 23(12): 5105 - 5112. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. L. Chi, S. Martinez, W. Wurst, and G. R. Martin The isthmic organizer signal FGF8 is required for cell survival in the prospective midbrain and cerebellum Development, June 15, 2003; 130(12): 2633 - 2644. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Brodski, D. M. V. Weisenhorn, M. Signore, I. Sillaber, M. Oesterheld, V. Broccoli, D. Acampora, A. Simeone, and W. Wurst Location and Size of Dopaminergic and Serotonergic Cell Populations Are Controlled by the Position of the Midbrain-Hindbrain Organizer J. Neurosci., May 15, 2003; 23(10): 4199 - 4207. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. Olivera-Martinez, S. Missier, S. Fraboulet, J. Thelu, and D. Dhouailly Differential regulation of the chick dorsal thoracic dermal progenitors from the medial dermomyotome Development, March 12, 2003; 129(20): 4763 - 4772. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Reim and M. Brand spiel-ohne-grenzen/pou2 mediates regional competence to respond to Fgf8 during zebrafish early neural development Development, March 4, 2003; 129(4): 917 - 933. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Kobayashi, M. Fujioka, E. N. Tolkunova, D. Deka, M. Abu-Shaar, R. S. Mann, and J. B. Jaynes Engrailed cooperates with extradenticle and homothorax to repress target genes in Drosophila Development, February 15, 2003; 130(4): 741 - 751. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Patel and A. J. Barkovich Analysis and Classification of Cerebellar Malformations AJNR Am. J. Neuroradiol., August 1, 2002; 23(7): 1074 - 1087. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Marie, L. Cruz-Orengo, and J. M. Blagburn Persistent engrailed Expression Is Required to Determine Sensory Axon Trajectory, Branching, and Target Choice J. Neurosci., February 1, 2002; 22(3): 832 - 841. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Chen and D. E. Harrison Quantitative trait loci regulating relative lymphocyte proportions in mouse peripheral blood Blood, January 15, 2002; 99(2): 561 - 566. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
O. Voiculescu, E. Taillebourg, C. Pujades, C. Kress, S. Buart, P. Charnay, and S. Schneider-Maunoury Hindbrain patterning: Krox20 couples segmentation and specification of regional identity Development, December 15, 2001; 128(24): 4967 - 4978. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H.-G. Belting, G. Hauptmann, D. Meyer, S. Abdelilah-Seyfried, A. Chitnis, C. Eschbach, I. Soll, C. Thisse, B. Thisse, K. B. Artinger, et al. spiel ohne grenzen/pou2 is required during establishment of the zebrafish midbrain-hindbrain boundary organizer Development, November 1, 2001; 128(21): 4165 - 4176. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Sato, I. Araki, and H. Nakamura Inductive signal and tissue responsiveness defining the tectum and the cerebellum Development, July 1, 2001; 128(13): 2461 - 2469. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Mahlapuu, S. Enerback, and P. Carlsson Haploinsufficiency of the forkhead gene Foxf1, a target for sonic hedgehog signaling, causes lung and foregut malformations Development, June 15, 2001; 128(12): 2397 - 2406. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. L. Montesinos, I. Foucher, M. Conradt, G. Mainguy, L. Robel, A. Prochiantz, and M. Volovitch The Neuronal Microtubule-Associated Protein 1B Is under Homeoprotein Transcriptional Control J. Neurosci., May 15, 2001; 21(10): 3350 - 3359. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. H. Simon, H. Saueressig, W. Wurst, M. D. Goulding, and D. D. M. O'Leary Fate of Midbrain Dopaminergic Neurons Controlled by the Engrailed Genes J. Neurosci., May 1, 2001; 21(9): 3126 - 3134. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V Brault, R Moore, S Kutsch, M Ishibashi, D. Rowitch, A. McMahon, L Sommer, O Boussadia, and R Kemler Inactivation of the (&bgr;)-catenin gene by Wnt1-Cre-mediated deletion results in dramatic brain malformation and failure of craniofacial development Development, January 4, 2001; 128(8): 1253 - 1264. [Abstract] [PDF]
|
|
|
|
|
|
A Liu and A. Joyner EN and GBX2 play essential roles downstream of FGF8 in patterning the mouse mid/hindbrain region Development, January 1, 2001; 128(2): 181 - 191. [Abstract] [PDF]
|
|
|
|
 |
|
 H. B. Sarnat Molecular Genetic Classification of Central Nervous System Malformations J Child Neurol, October 1, 2000; 15(10): 675 - 687. [Abstract] [PDF]
|
|
|
|
|
|
L. Studer, M. Csete, S.-H. Lee, N. Kabbani, J. Walikonis, B. Wold, and R. McKay Enhanced Proliferation, Survival, and Dopaminergic Differentiation of CNS Precursors in Lowered Oxygen J. Neurosci., October 1, 2000; 20(19): 7377 - 7383. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. A. Kimmel, D. H. Turnbull, V. Blanquet, W. Wurst, C. A. Loomis, and A. L. Joyner Two lineage boundaries coordinate vertebrate apical ectodermal ridge formation Genes & Dev., June 1, 2000; 14(11): 1377 - 1389. [Abstract] [Full Text]
|
|
|
|
|
|
H. B. Samat and J. H. Menkes How to Construct a Neural Tube J Child Neurol, February 1, 2000; 15(2): 110 - 124. [PDF]
|
|
|
|
|
|
A Louvi and M Wassef Ectopic engrailed 1 expression in the dorsal midline causes cell death, abnormal differentiation of circumventricular organs and errors in axonal pathfinding Development, January 9, 2000; 127(18): 4061 - 4071. [Abstract] [PDF]
|
|
|
|
|
|
E Matsunaga, I Araki, and H Nakamura Pax6 defines the di-mesencephalic boundary by repressing En1 and Pax2 Development, January 6, 2000; 127(11): 2357 - 2365. [Abstract] [PDF]
|
|
|
|
|
|
K. Adams, J. Maida, J. Golden, and R. Riddle The transcription factor Lmx1b maintains Wnt1 expression within the isthmic organizer Development, January 5, 2000; 127(9): 1857 - 1867. [Abstract] [PDF]
|
|
|
|
|
|
P. Pfeffer, M Bouchard, and M Busslinger Pax2 and homeodomain proteins cooperatively regulate a 435 bp enhancer of the mouse Pax5 gene at the midbrain-hindbrain boundary Development, January 3, 2000; 127(5): 1017 - 1028. [Abstract] [PDF]
|
|
|
|
|
|
Y Watanabe and H Nakamura Control of chick tectum territory along dorsoventral axis by Sonic hedgehog Development, January 3, 2000; 127(5): 1131 - 1140. [Abstract] [PDF]
|
|
|
|
|
|
P. F. Chance, L. Cavalier, D. Satran, J. E. Pellegrino, M. Koenig, and W. B. Dobyns Clinical Nosologic and Genetic Aspects of Joubert and Related Syndromes J Child Neurol, October 1, 1999; 14(10): 660 - 666. [Abstract] [PDF]
|
|
|
|
|
|
A. T. Yachnis and L. B. Rorke Cerebellar and Brainstem Development: An Overview in Relation to Joubert Syndrome J Child Neurol, September 1, 1999; 14(9): 570 - 573. [Abstract] [PDF]
|
|
|
|
|
|
S. L. Baader, M. W. Vogel, S. Sanlioglu, X. Zhang, and J. Oberdick Selective Disruption of "Late Onset" Sagittal Banding Patterns by Ectopic Expression of Engrailed-2 in Cerebellar Purkinje Cells J. Neurosci., July 1, 1999; 19(13): 5370 - 5379. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. L. Maria, R. G. Quisling, L. C. Rosainz, A. T. Yachnis, J. Gitten, D. Dede, and E. Fennell Molar Tooth Sign in Joubert Syndrome: Clinical, Radiologic, and Pathologic Significance J Child Neurol, June 1, 1999; 14(6): 368 - 376. [Abstract] [PDF]
|
|
|
|
 |
|
 J. Gemel, C. Jacobsen, and C. A. MacArthur Fibroblast Growth Factor-8 Expression Is Regulated by Intronic Engrailed and Pbx1-binding Sites J. Biol. Chem., February 26, 1999; 274(9): 6020 - 6026. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L I Al-Gazali, L Sztriha, J Punnose, W Shather, and M Nork Absent pituitary gland and hypoplasia of the cerebellar vermis associated with partial ophthalmoplegia and postaxial polydactyly: a variant of orofaciodigital syndrome VI or a new syndrome? J. Med. Genet., February 1, 1999; 36(2): 161 - 166. [Abstract] [Full Text]
|
|
|
|
|
|
M Carl and J Wittbrodt Graded interference with FGF signalling reveals its dorsoventral asymmetry at the mid-hindbrain boundary Development, January 12, 1999; 126(24): 5659 - 5667. [Abstract] [PDF]
|
|
|
|
|
|
A Liu, K Losos, and A. Joyner FGF8 can activate Gbx2 and transform regions of the rostral mouse brain into a hindbrain fate Development, January 11, 1999; 126(21): 4827 - 4838. [Abstract] [PDF]
|
|
|
|
|
|
I Araki and H Nakamura Engrailed defines the position of dorsal di-mesencephalic boundary by repressing diencephalic fate Development, January 11, 1999; 126(22): 5127 - 5135. [Abstract] [PDF]
|
|
|
|
|
|
H Saueressig, J Burrill, and M Goulding Engrailed-1 and netrin-1 regulate axon pathfinding by association interneurons that project to motor neurons Development, January 10, 1999; 126(19): 4201 - 4212. [Abstract] [PDF]
|
|
|
|
|
|
F Ristoratore, M Carl, K Deschet, L Richard-Parpaillon, D Boujard, J Wittbrodt, D Chourrout, F Bourrat, and J. Joly The midbrain-hindbrain boundary genetic cascade is activated ectopically in the diencephalon in response to the widespread expression of one of its components, the medaka gene Ol-eng2 Development, January 9, 1999; 126(17): 3769 - 3779. [Abstract] [PDF]
|
|
|
|
|
|
A Picker, C Brennan, F Reifers, J. Clarke, N Holder, and M Brand Requirement for the zebrafish mid-hindbrain boundary in midbrain polarisation, mapping and confinement of the retinotectal projection Development, January 7, 1999; 126(13): 2967 - 2978. [Abstract] [PDF]
|
|
|
|
|
|
S Martinez, P. Crossley, I Cobos, J. Rubenstein, and G. Martin FGF8 induces formation of an ectopic isthmic organizer and isthmocerebellar development via a repressive effect on Otx2 expression Development, January 3, 1999; 126(6): 1189 - 1200. [Abstract] [PDF]
|
|
|
|
|
|
H Shamim, R Mahmood, C Logan, P Doherty, A Lumsden, and I Mason Sequential roles for Fgf4, En1 and Fgf8 in specification and regionalisation of the midbrain Development, January 2, 1999; 126(5): 945 - 959. [Abstract] [PDF]
|
|
|
|
|
|
S. L. Baader, S. Sanlioglu, A. S. Berrebi, J. Parker-Thornburg, and J. Oberdick Ectopic Overexpression of Engrailed-2 in Cerebellar Purkinje Cells Causes Restricted Cell Loss and Retarded External Germinal Layer Development at Lobule Junctions J. Neurosci., March 1, 1998; 18(5): 1763 - 1773. [Abstract] |
