Genomes and Genes
Gene Symbol: Heyl
Description: hairy/enhancer-of-split related with YRPW motif-like
Alias: Hey3, Hrt3, bHLHb33, hesr3, hairy/enhancer-of-split related with YRPW motif-like protein, HRT-3, hairy and enhance of split related 3, hairy and enhancer of split-related protein 3, hairy-related transcription factor 3, mHRT3
- RBPjkappa-dependent Notch signaling regulates mesenchymal progenitor cell proliferation and differentiation during skeletal developmentYufeng Dong
Department of Orthopaedics and Rehabilitation, Center for Musculoskeletal Research, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA
Development 137:1461-71. 2010..Finally, Hes1 was identified as an RBPjkappa-dependent Notch target gene important for MPC maintenance and the suppression of in vitro chondrogenesis...
- Inactivation of Notch signaling in the renal collecting duct causes nephrogenic diabetes insipidus in miceHyun Woo Jeong
Department of Biological Sciences, Seoul National University, Seoul, Republic of Korea
J Clin Invest 119:3290-300. 2009..Our data demonstrate that Notch signaling is required for the development of the mammalian renal collecting duct and principal cell differentiation and indicate that pathway dysregulation may contribute to distal renal tubular disorders...
- HeyL promotes neuronal differentiation of neural progenitor cellsAli Jalali
Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
J Neurosci Res 89:299-309. 2011..We found that HeyL, contrary to the classic function of Hes and Hey factors, promotes neuronal differentiation of neural progenitor ..
- Hesr1 and Hesr3 are essential to generate undifferentiated quiescent satellite cells and to maintain satellite cell numbersSo ichiro Fukada
Department of Immunology, Graduate School of Pharmaceutical Sciences, Osaka University, 1 6 Yamada oka, Suita, Osaka 565 0871, Japan
Development 138:4609-19. 2011..We show that Hesr1 (Hey1) and Hesr3 (Heyl) (which are known Notch target genes) are expressed simultaneously in skeletal muscle only in satellite ..
- Notch signaling maintains bone marrow mesenchymal progenitors by suppressing osteoblast differentiationMatthew J Hilton
Department of Medicine, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, Missouri 63110, USA
Nat Med 14:306-14. 2008..Thus, mesenchymal progenitors may be expanded in vitro by activating the Notch pathway, whereas bone formation in vivo may be enhanced by transiently suppressing this pathway...
- Combined loss of Hey1 and HeyL causes congenital heart defects because of impaired epithelial to mesenchymal transitionAndreas Fischer
Department of Physiological Chemistry I, Biocenter, University of Wurzburg, Wurzburg, Germany
Circ Res 100:856-63. 2007..Here we report that combined inactivation of Hey1 and HeyL, two primary target genes of Notch, causes severe heart malformations, including membranous ventricular septal ..
- Hey basic helix-loop-helix transcription factors are repressors of GATA4 and GATA6 and restrict expression of the GATA target gene ANF in fetal heartsAndreas Fischer
Theodor Boveri Institute, Physiological Chemistry I, University of Wuerzburg, Germany
Mol Cell Biol 25:8960-70. 2005..In addition, the promoter activity of the GATA4/6 target gene ANF was inhibited by Hey1, Hey2, and HeyL. Protein interaction and mutation analyses suggest that repression is due to direct binding of Hey proteins to ..
- Context-Dependent Sensitivity to Mutations Disrupting the Structural Integrity of Individual EGF Repeats in the Mouse Notch Ligand DLL1Karin Schuster-Gossler
Institut fur Molekularbiologie, Medizinische Hochschule Hannover, 30625 Hannover, Germany
Genetics 202:1119-33. 2016..In conclusion, the structural integrity of each individual EGF repeat in the extracellular domain of DLL1 is necessary for full DLL1 activity, and certain mutations in Dll1 might contribute to spondylocostal dysostosis in humans. ..
- Notch activation induces endothelial cell senescence and pro-inflammatory response: implication of Notch signaling in atherosclerosisZhao Jun Liu
Department of Surgery, University of Miami, Miller School of Medicine, University of Miami, Miami, FL 33136, USA
Atherosclerosis 225:296-303. 2012..Notch signaling plays pivotal roles in the pathogenesis of vascular disease. However, little is known about its role in atherosclerosis. We sought to investigate the potential involvement of the Notch signaling in atherosclerosis...
- Disruption of sorting nexin 5 causes respiratory failure associated with undifferentiated alveolar epithelial type I cells in miceSun Kyoung Im
School of Biological Science, College of Natural Sciences, Seoul National University, Seoul, South Korea
PLoS ONE 8:e58511. 2013..These results demonstrate that Snx5 is necessary for the differentiation of alveolar epithelial type I cells, which may underlie the adaptation to air breathing at birth...
- Notch/Rbpjκ signaling regulates progenitor maintenance and differentiation of hypothalamic arcuate neuronsPaven K Aujla
University of Illinois at Urbana Champaign, 407 South Goodwin Avenue, Urbana, IL 61801, USA
Development 140:3511-21. 2013..Taken together, our results demonstrate that Notch/Rbpjκ signaling regulates the generation and differentiation of Arc neurons, which contribute to homeostatic regulation of body size. ..
- Regulation of mesenchymal-to-epithelial transition by PARAXIS during somitogenesisMegan Rowton
School of Life Sciences, Arizona State University, Tempe, Arizona
Dev Dyn 242:1332-44. 2013..However, the target genes downstream of the transcription factor PARAXIS remain poorly described...
- Transcription factor Hes1 modulates osteoarthritis development in cooperation with calcium/calmodulin-dependent protein kinase 2Shurei Sugita
Sensory and Motor System Medicine
Proc Natl Acad Sci U S A 112:3080-5. 2015..Our findings have contributed to further understanding of the molecular pathophysiology of OA, and may provide the basis for development of novel treatments for joint disorders. ..
- Ephrin-B2 governs morphogenesis of endolymphatic sac and duct epithelia in the mouse inner earSteven Raft
Section on Sensory Cell Regeneration and Development, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA Electronic address
Dev Biol 390:51-67. 2014..We propose that developmental dysplasias described here are a gene dose-sensitive cause of the vestibular dysfunction observed in EphB-Efnb2 signaling-deficient mice. ..
- Fibroblast growth factor receptor-Frs2α signaling is critical for nephron progenitorsValeria Di Giovanni
Division of Nephrology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA 15201, USA
Dev Biol 400:82-93. 2015..Thus, Fgfr1 and Fgfr2 have synergistic roles in maintaining nephron progenitors; furthermore, Fgfr signaling in nephron progenitors appears to be mediated predominantly by Frs2α. ..
- The Notch pathway inhibits TGFβ signaling in breast cancer through HEYL-mediated crosstalkLiangfeng Han
Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland
Cancer Res 74:6509-18. 2014..Here, we present evidence of crosstalk between these two pathways through HEYL. HEYL, a basic helix-loop-helix transcription factor and a direct target of Notch signaling, is specifically ..
- RBPJ in mouse Sertoli cells is required for proper regulation of the testis stem cell nicheThomas Xavier Garcia
Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Unit 1105, PO Box 301402, Houston, TX 77230 1402, USA Department of Comparative Biosciences, University of Illinois at Urbana Champaign, Urbana, IL 61802, USA
Development 141:4468-78. 2014....
- Mutations in the NOTCH pathway regulator MIB1 cause left ventricular noncompaction cardiomyopathyGuillermo Luxan
Program of Cardiovascular Developmental Biology, Department of Cardiovascular Development and Repair, Centro Nacional de Investigaciones Cardiovasculares, Madrid, Spain
Nat Med 19:193-201. 2013..These results implicate NOTCH signaling in LVNC and indicate that MIB1 mutations arrest chamber myocardium development, preventing trabecular maturation and compaction...
- Numb deletion in POMC-expressing cells impairs pituitary intermediate lobe cell adhesion, progenitor cell localization, and neuro-intermediate lobe boundary formationTyler B Moran
University of Illinois at Urbana Champaign, 407 South Goodwin Avenue, Urbana, Illinois 61801, USA
Mol Endocrinol 25:117-27. 2011..Unexpectedly, Notch activity appears normal in conditional knockout mice. Thus, Numb is critical for maintaining cell-cell interactions in the pituitary intermediate lobe that are essential for proper cell placement...
- Mastermind-like 1 (MamL1) and mastermind-like 3 (MamL3) are essential for Notch signaling in vivoToshinao Oyama
Department of Molecular and Tumor Pathology, Chiba University Graduate School of Medicine, 1 8 1 Inohana, Chuo Ku, Chiba 260 8670, Japan
Development 138:5235-46. 2011..These results indicate that engagement of Mam is essential for Notch signaling, and that the three Mam isoforms have distinct roles in vivo...
- Physiological notch signaling maintains bone homeostasis via RBPjk and Hey upstream of NFATc1Xiaolin Tu
Department of Medicine, Washington University School of Medicine, St Louis, Missouri, United States of America
PLoS Genet 8:e1002577. 2012..Moreover, mice deficient in Hey1 and HeyL, two target genes of Notch-RBPjk signaling, exhibited high bone mass...
- Target gene analysis by microarrays and chromatin immunoprecipitation identifies HEY proteins as highly redundant bHLH repressorsJulia Heisig
Developmental Biochemistry, Theodor Boveri Institute, Biocenter, University of Wuerzburg, Wuerzburg, Germany
PLoS Genet 8:e1002728. 2012..The striking overlap of cardiac defects in HEY2 and combined HEY1/HEYL knockout mice suggested that all three HEY genes fulfill overlapping function in target cells...
- Persistent expression of activated Notch inhibits corticotrope and melanotrope differentiation and results in dysfunction of the HPA axisLeah B Goldberg
Department of Molecular and Integrative Physiology, University of Illinois at Urbana Champaign, Urbana, IL 61801, USA
Dev Biol 358:23-32. 2011..Taken together, these findings show that Notch signaling is sufficient to prevent corticotrope and melanotrope differentiation, resulting in dysregulation of the HPA axis...
- Notch signaling regulates remodeling and vessel diameter in the extraembryonic yolk sacJessica N Copeland
Department of Pathology and Laboratory Medicine and Institute for Reproductive Health and Regenerative Medicine, University of Kansas Medical Center, Kansas City, KS 66160, USA
BMC Dev Biol 11:12. 2011..To further understand the role of Notch signaling during endothelial differentiation and the genes regulated by this pathway, both loss-of-function and gain-of-function approaches were analyzed in vivo...
- Cooperative functions of Hes/Hey genes in auditory hair cell and supporting cell developmentTomoko Tateya
Institute for Virus Research, Kyoto University, Kyoto 606 8507, Japan
Dev Biol 352:329-40. 2011....
- Differential effects of the HESR/HEY transcription factor family on dopamine transporter reporter gene expression via variable number of tandem repeatsKouta Kanno
Department of Life Sciences, Graduate School of Arts and Sciences, The University of Tokyo, Tokyo, Japan
J Neurosci Res 89:562-75. 2011..Other members of the HESR (HEY) family, including HESR2 (HEY2) and 3 (HEYL), have similar DNA-binding domains...
- Numb promotes an increase in skeletal muscle progenitor cells in the embryonic somiteAurélie Jory
Stem Cells and Development, Department of Developmental Biology Pasteur Institute, Centre National de la Recherche Scientifique CNRS URA 2578, 75724 Paris Cedex 15, France
Stem Cells 27:2769-80. 2009..Thus, we propose that Numb can regulate the self-renewal of dermal and muscle progenitors during a lineage progression...
- Segmental expression of Notch and Hairy genes in nephrogenesisLinghong Chen
Department of Medicine, College of Physicians and Surgeons of Columbia University, 630 W 168th St, New York, NY 10032, USA
Am J Physiol Renal Physiol 288:F939-52. 2005..We found that among all of Notch downstream Hairy genes, only Hes1, Hes5, Hey1, and HeyL were expressed in a segment-specific manner in early nephrons and their expression pattern changed dynamically ..
- HRT1, HRT2, and HRT3: a new subclass of bHLH transcription factors marking specific cardiac, somitic, and pharyngeal arch segmentsO Nakagawa
Department of Molecular Biology, The University of Texas Southwestern Medical Center at Dallas, 6000 Harry Hines Boulevard, Dallas, Texas, 75235 9148, USA
Dev Biol 216:72-84. 1999..Here, we describe a new subclass of bHLH proteins, HRT1 (Hairy-related transcription factor 1), HRT2, and HRT3, that share high homology with the Hairy family of proteins yet have characteristics that are distinct from those ..
- Characterization of the human and mouse HEY1, HEY2, and HEYL genes: cloning, mapping, and mutation screening of a new bHLH gene familyC Steidl
Physiologische Chemie I, Biozentrum der Universität Würzburg, Am Hubland, Wurzburg, 97074, Germany
Genomics 66:195-203. 2000..factor genes from mouse and human (hairy and Enhancer-of-split related with YRPW motif; HEY1, HEY2, and HEYL). All three HEY genes have a similar genomic structure with five exons...
- The basic helix-loop-helix transcription factors dHAND and eHAND exhibit dimerization characteristics that suggest complex regulation of functionB A Firulli
Department of Physiology, The University of Texas Health Science Center at San Antonio, San Antonio, Texas 78229 3900, USA
J Biol Chem 275:33567-73. 2000..Taken together, these results show that dHAND and eHAND can form homo- and heterodimer combinations with multiple bHLH partners and that this broad dimerization profile reflects the mechanisms by which HAND genes regulate transcription...
- Analysis of HeyL expression in wild-type and Notch pathway mutant mouse embryosC Leimeister
Institute of Physiological Chemistry I, Theodor Boveri Institute Biocenter, University of Wuerzburg, Am Hubland, D 97074, Wuerzburg, Germany
Mech Dev 98:175-8. 2000..Here we report the embryonic expression of the hairy-related basic helix-loop-helix gene HeyL in wild-type and Notch pathway mutant mice...
- Members of the HRT family of basic helix-loop-helix proteins act as transcriptional repressors downstream of Notch signalingO Nakagawa
Departments of Molecular Biology and Pediatrics, The University of Texas Southwestern Medical Center at Dallas, 6000 Harry Hines Boulevard, Dallas, Texas 75390 9148, USA
Proc Natl Acad Sci U S A 97:13655-60. 2000..These findings identify HRT genes as downstream targets for Notch signaling and reveal a negative autoregulatory loop whereby HRT proteins repress their own expression through interference with Notch signaling...
- The basic helix-loop-helix gene hesr2 promotes gliogenesis in mouse retinaT Satow
Institute for Virus Research, Kyoto University, and Department of Neurosurgery, Kyoto University Graduate School of Medicine, Sakyo ku, Kyoto 606 8507, Japan
J Neurosci 21:1265-73. 2001..Interestingly, hesr2 but not hesr1 or hesr3 promoted gliogenesis while inhibiting rod genesis without affecting cell proliferation or death, suggesting that ..
- Expression of Notch pathway genes in the embryonic mouse metanephros suggests a role in proximal tubule developmentCornelia Leimeister
Theodor Boveri Institute, Biozentrum, Physiological Chemistry I, University of Wuerzburg, Am Hubland, 97074, Wuerzburg, Germany
Gene Expr Patterns 3:595-8. 2003..Our results point to a Lfng-dependent role for Notch signalling in the development of nephron segments, especially the proximal tubules...
- Mouse Fbw7/Sel-10/Cdc4 is required for notch degradation during vascular developmentRyosuke Tsunematsu
Department of Molecular and Cellular Biology, Medical Institute of Bioregulation, Kyushu University, Fukuoka, Japan
J Biol Chem 279:9417-23. 2004..Expression of Notch1, -2, or -3 or of cyclin E was unaffected in Fbw7(-/-) embryos. Mammalian Fbw7 thus appears to play an indispensable role in negative regulation of the Notch4-Hey1 pathway and is required for vascular development...
- Gene structure and evolution of testicular haploid germ cell-specific genes, Oxct2a and Oxct2bMasayoshi Onishi
Department of Science for Laboratory Animal Experimentation, Research Institute for Microbial Diseases, Osaka University, 3 1 Yamadaoka, Suita City, Osaka 565 0871, Japan
Genomics 83:647-57. 2004..Dot matrix and phylogenetic tree analyses demonstrated that multiple rounds of intrachromosomal gene conversion between the two loci occurred in each species independently...
- Dll3 pudgy mutation differentially disrupts dynamic expression of somite genesKenro Kusumi
Divisions of Human Genetics and Orthopaedic Surgery, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
Genesis 39:115-21. 2004..the Dll3(pu) mutation has different effects on the expression of cycling (Lfng and Hes7) and stage-specific genes (Hey3 and Mesp2)...
- Hey genes: a novel subfamily of hairy- and Enhancer of split related genes specifically expressed during mouse embryogenesisC Leimeister
Institute of Physiological Chemistry I, Theodor Boveri Institute Biocenter, University of Wuerzburg, Am Hubland, D 97074, Wuerzburg, Germany
Mech Dev 85:173-7. 1999..The diversity of expression patterns implies unique functions in neurogenesis, somitogenesis and organogenesis...
- Premature myogenic differentiation and depletion of progenitor cells cause severe muscle hypotrophy in Delta1 mutantsKarin Schuster-Gossler
Institute for Molecular Biology, Medizinische Hochschule Hannover, Carl Neuberg Strasse 1, Hannover, D 30625 Lower Saxony, Germany
Proc Natl Acad Sci U S A 104:537-42. 2007....
- An essential role for Notch in neural crest during cardiovascular development and smooth muscle differentiationFrances A High
Cardiovascular Institute, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
J Clin Invest 117:353-63. 2007..These results provide a molecular and cellular framework for understanding the role of Notch signaling in the etiology of congenital heart disease...
- Hesr1 and Hesr2 may act as early effectors of Notch signaling in the developing cochleaToshinori Hayashi
Virginia Merrill Bloedel Hearing Research Center, Department of Otolaryngology HNS, Box 357923, USA
Dev Biol 316:87-99. 2008..This treatment also reduces Hesr1 and Hesr2 expression by as much as 80%. These results support the hypothesis that Hesr1 and Hesr2 are the downstream mediators of the prosensory function of Notch in early cochlear development...
- Notch signalling in the paraxial mesoderm is most sensitive to reduced Pofut1 levels during early mouse developmentKarin Schuster-Gossler
Institute for Molecular Biology, Medizinische Hochschule Hannover, Carl Neuberg Str, 1, D 30625, Germany
BMC Dev Biol 9:6. 2009..Whether the context-dependent effects of Notch receptor glycosylation by FNG reflect different requirements of distinct developmental processes for O-fucosylation by POFUT1 is unclear...
- LGR5 deficiency deregulates Wnt signaling and leads to precocious Paneth cell differentiation in the fetal intestineMarie Isabelle Garcia
Institut de Recherche Interdisciplinaire en Biologie Humaine et Moléculaire IRIBHM, Faculty of Medicine, Universite Libre de Bruxelles, 1070 Brussels, Belgium
Dev Biol 331:58-67. 2009..Together, our data identify LGR5 as a negative regulator of the Wnt pathway in the developing intestine...
- Jagged1 is a competitive inhibitor of Notch signaling in the embryonic pancreasMaria L Golson
Department of Genetics, School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
Mech Dev 126:687-99. 2009..Expression of the Notch modifier Manic Fringe (Mfng) is limited to endocrine precursors, providing a possible explanation for the inhibition of Notch signaling by Jag1 during mid-gestation embryonic pancreas development...
- HeyL regulates the number of TrkC neurons in dorsal root gangliaAbhishek Mukhopadhyay
Department of Neurology, Northwestern University s Feinberg School of Medicine, Chicago, IL 60611, USA
Dev Biol 334:142-51. 2009The basic-helix-loop-helix transcription factor HeyL is expressed at high levels by neural crest progenitor cells (NCPs) that give rise to neurons and glia in dorsal root ganglia (DRG)...
- Fibroblast growth factor 9 signaling inhibits airway smooth muscle differentiation in mouse lungLan Yi
Laboratory of Genetics, University of Wisconsin Madison, Madison, Wisconsin 53706, USA
Dev Dyn 238:123-37. 2009..This model also represents our findings on the genetic relationship between FGF9 and sonic hedgehog (SHH) in the establishment of airway SMC pattern...
- Notch signaling controls liver development by regulating biliary differentiationYiwei Zong
Abramson Family Cancer Research Institute, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
Development 136:1727-39. 2009..These results reconcile previous conflicting reports about the role of Notch during liver development and suggest that Notch acts by coordinating biliary differentiation and morphogenesis...
- Phenotypic screen in mouse embryonic for TGF-beta signaling mutationsJAY VIVIAN; Fiscal Year: 2007..Cell-based mutation screening strategies as developed in this proposal can be applied to any active biochemical pathway in mouse ES cells, to generate mouse lines for analysis in vivo. [unreadable] [unreadable] [unreadable]..