nebulette

Summary

Gene Symbol: nebulette
Description: nebulette
Alias: LASP2, LNEBL, nebulette, LIM and SH3 protein 2, LIM-nebulette, actin-binding Z-disk protein
Species: human

Top Publications

  1. ncbi Zyxin interacts with the SH3 domains of the cytoskeletal proteins LIM-nebulette and Lasp-1
    Bo Li
    ITI Research Institute, University of Bern, P O Box 54, CH 3010 Bern, Switzerland
    J Biol Chem 279:20401-10. 2004
  2. pmc Nebulette mutations are associated with dilated cardiomyopathy and endocardial fibroelastosis
    Enkhsaikhan Purevjav
    The Heart Institute, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    J Am Coll Cardiol 56:1493-502. 2010
  3. pmc Ectopic expression of LIM-nebulette (LASP2) reveals roles in cell migration and spreading
    Xiaodi A Deng
    Department of Molecular and Cellular Biochemistry, University of Kentucky, Lexington, Kentucky 40536, USA
    Cell Motil Cytoskeleton 65:827-40. 2008
  4. ncbi Identification and characterization of LASP2 gene in silico
    Masuko Katoh
    M and M Medical BioInformatics, Narashino 275 0022, Japan
    Int J Mol Med 12:405-10. 2003
  5. ncbi Targeted disruption of nebulette protein expression alters cardiac myofibril assembly and function
    Carole L Moncman
    Department of Chemistry and Biochemistry, University of Texas at Austin, Austin, Texas 78712, USA
    Exp Cell Res 273:204-18. 2002
  6. pmc Myopalladin, a novel 145-kilodalton sarcomeric protein with multiple roles in Z-disc and I-band protein assemblies
    M L Bang
    European Molecular Biology Laboratory, Heidelberg 69117, Germany
    J Cell Biol 153:413-27. 2001
  7. ncbi Nebulette: a 107 kD nebulin-like protein in cardiac muscle
    C L Moncman
    Department of Chemistry and Biochemistry, University of Texas, Austin 78712, USA
    Cell Motil Cytoskeleton 32:205-25. 1995
  8. ncbi Functional dissection of nebulette demonstrates actin binding of nebulin-like repeats and Z-line targeting of SH3 and linker domains
    C L Moncman
    Department of Chemistry and Biochemistry, University of Texas at Austin, Austin, USA
    Cell Motil Cytoskeleton 44:1-22. 1999
  9. ncbi Characterization of nebulette and nebulin and emerging concepts of their roles for vertebrate Z-discs
    S Millevoi
    Department of Structural Biology, Meyerhofstr 1, EMBL Heidelberg, 69012, Germany stefania Millevoi EMBL Heidelberg de
    J Mol Biol 282:111-23. 1998
  10. ncbi Linker region of nebulin family members plays an important role in targeting these molecules to cellular structures
    Zivile Panaviene
    Department of Molecular and Cellular Biochemistry, University of Kentucky College of Medicine, Lexington, KY 40536, USA
    Cell Tissue Res 327:353-69. 2007

Scientific Experts

  • Gillian C Barnett
  • Audrey V Grant
  • Florence Pedeutour
  • Thomas Gp Grunewald
  • Carole L Moncman
  • Andreas Perrot
  • Ho Chang Kuo
  • Zivile Panaviene
  • Sae Mi Yoo
  • Giuseppina Mastrototaro
  • Shyam S Jayaraman
  • K Wachter
  • Mariana Emerenciano
  • Stefan Eulitz
  • Katherine T Bliss
  • K Maiellaro-Rafferty
  • Rolf Marschalek
  • Claus Meyer
  • Maria S Pombo-de-Oliveira
  • Enkhsaikhan Purevjav
  • Virginia M Coser
  • Jing Miao
  • Marc Weinkauf
  • Xiaodi A Deng
  • William B Holmes
  • Jeremy R Bonzo
  • Nicholas I Proellocks
  • Ming Lu
  • Bo Li
  • S A Yatsenko
  • C L Moncman
  • Yu Chen
  • Wen Ching Chan
  • Pascale Richard
  • Silke R Sperling
  • Ying Hsien Huang
  • Janine Lossie
  • Hsing Chun Kuo
  • Jorge Andrade
  • Marzia De Bortoli
  • Nadine Lohmann
  • Hong Ren Yu
  • Mindy Ming Huey Guo
  • Akos Varga-Szemes
  • Fu Chen Huang
  • Fuyong Jiao
  • Paola Melacini
  • Annalisa Angelini
  • Josef Veselka
  • Reka Faludi
  • Philippe Charron
  • Masuko Katoh
  • Tamas Simor
  • Sung Chou Li
  • Cemil Ozcelik
  • Pavol Tomasov
  • Eric Villard
  • Sung Hyun Choi
  • Chiara Tesi
  • Yusu Gu
  • Nancy D Dalton
  • Farah Sheikh
  • Ju Chen
  • Xingqun Liang
  • Xiaodong Li
  • Sang Hong Baek
  • Corrado Poggesi
  • Monica Dha Yea Jung
  • Sung Cil Lim
  • Marie Louise Bang
  • Nicoletta Piroddi
  • Kirk L Peterson
  • Pierluigi Carullo
  • David J Rayhan
  • Salar Hazany
  • E Kowarz
  • Michael S Kolodney
  • R Marschalek
  • Melissa P Villanueva
  • Silvia Bracharz
  • J P Wansapura
  • Peter F M van der Ven
  • Julia Schuld
  • Colin M Jones-Weinert
  • Florian Sauer
  • J F James
  • Gregor Kirfel
  • J Robbins
  • Eric Kowarz
  • Miensheng Chu

Detail Information

Publications35

  1. ncbi Zyxin interacts with the SH3 domains of the cytoskeletal proteins LIM-nebulette and Lasp-1
    Bo Li
    ITI Research Institute, University of Bern, P O Box 54, CH 3010 Bern, Switzerland
    J Biol Chem 279:20401-10. 2004
    ..Here we describe a novel binding partner of zyxin, which we have named LIM-nebulette. LIM-nebulette is an alternative splice variant of the sarcomeric protein nebulette, which, in contrast to ..
  2. pmc Nebulette mutations are associated with dilated cardiomyopathy and endocardial fibroelastosis
    Enkhsaikhan Purevjav
    The Heart Institute, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    J Am Coll Cardiol 56:1493-502. 2010
    Four variants (K60N, Q128R, G202R, and A592E) in the nebulette gene were identified in patients with dilated cardiomyopathy (DCM) and endocardial fibroelastosis. We sought to determine if these mutations are cardiomyopathy causing.
  3. pmc Ectopic expression of LIM-nebulette (LASP2) reveals roles in cell migration and spreading
    Xiaodi A Deng
    Department of Molecular and Cellular Biochemistry, University of Kentucky, Lexington, Kentucky 40536, USA
    Cell Motil Cytoskeleton 65:827-40. 2008
    LIM-nebulette (LASP2) is a small focal adhesion protein and a member of the nebulin family of actin binding proteins...
  4. ncbi Identification and characterization of LASP2 gene in silico
    Masuko Katoh
    M and M Medical BioInformatics, Narashino 275 0022, Japan
    Int J Mol Med 12:405-10. 2003
    ..Here, we identified and characterized a novel LASP1-related gene, LASP2, by using bioinformatics...
  5. ncbi Targeted disruption of nebulette protein expression alters cardiac myofibril assembly and function
    Carole L Moncman
    Department of Chemistry and Biochemistry, University of Texas at Austin, Austin, Texas 78712, USA
    Exp Cell Res 273:204-18. 2002
    To evaluate nebulette's role in cardiac myofibrils, cardiomyocytes expressing green fluorescent protein (GFP)-nebulette constructs were monitored for their ability to contract and myofilament protein distribution was analyzed...
  6. pmc Myopalladin, a novel 145-kilodalton sarcomeric protein with multiple roles in Z-disc and I-band protein assemblies
    M L Bang
    European Molecular Biology Laboratory, Heidelberg 69117, Germany
    J Cell Biol 153:413-27. 2001
    ..145-kD protein, myopalladin, which tethers together the COOH-terminal Src homology 3 domains of nebulin and nebulette with the EF hand motifs of alpha-actinin in vertebrate Z-lines...
  7. ncbi Nebulette: a 107 kD nebulin-like protein in cardiac muscle
    C L Moncman
    Department of Chemistry and Biochemistry, University of Texas, Austin 78712, USA
    Cell Motil Cytoskeleton 32:205-25. 1995
    ..To distinguish this petite nebulin-like protein from the 600-900 kD skeletal muscle nebulin, we have named it nebulette.
  8. ncbi Functional dissection of nebulette demonstrates actin binding of nebulin-like repeats and Z-line targeting of SH3 and linker domains
    C L Moncman
    Department of Chemistry and Biochemistry, University of Texas at Austin, Austin, USA
    Cell Motil Cytoskeleton 44:1-22. 1999
    b>Nebulette, a 107 kDa protein associated with the I-Z-I complex of cardiac myofibrils, may play an important role in the assembly of the Z-line...
  9. ncbi Characterization of nebulette and nebulin and emerging concepts of their roles for vertebrate Z-discs
    S Millevoi
    Department of Structural Biology, Meyerhofstr 1, EMBL Heidelberg, 69012, Germany stefania Millevoi EMBL Heidelberg de
    J Mol Biol 282:111-23. 1998
    ..Recently, a 100 kDa nebulin-like protein has been described in the avian cardiac muscle and referred to as nebulette. We have determined the full-length (8 kb) cDNA sequence of the human nebulette...
  10. ncbi Linker region of nebulin family members plays an important role in targeting these molecules to cellular structures
    Zivile Panaviene
    Department of Molecular and Cellular Biochemistry, University of Kentucky College of Medicine, Lexington, KY 40536, USA
    Cell Tissue Res 327:353-69. 2007
    ..The family members now include nebulin, nebulette, N-RAP, LASP-1, and LIM-nebulette...
  11. pmc Genome-Wide Association Study Identifies Novel Susceptibility Genes Associated with Coronary Artery Aneurysm Formation in Kawasaki Disease
    Ho Chang Kuo
    Department of Pediatrics and Kawasaki Disease Center, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan
    PLoS ONE 11:e0154943. 2016
    ..Our results help to depict the potential routes of the pathogenesis of CAAs in KD patients and will facilitate researchers to improve the diagnosis and prognosis of KD in personalized medicine. ..
  12. pmc Nebulette knockout mice have normal cardiac function, but show Z-line widening and up-regulation of cardiac stress markers
    Giuseppina Mastrototaro
    Humanitas Clinical and Research Center, Via Manzoni 113, 20089 Rozzano, Milan, Italy University of Milano Bicocca, Milan, Italy
    Cardiovasc Res 107:216-25. 2015
    b>Nebulette is a 109 kDa modular protein localized in the sarcomeric Z-line of the heart...
  13. pmc Mutations in NEBL encoding the cardiac Z-disk protein nebulette are associated with various cardiomyopathies
    Andreas Perrot
    Charite Universitatsmedizin Berlin, Cardiovascular Genetics, Experimental and Clinical Research Center, Berlin, Germany
    Arch Med Sci 12:263-78. 2016
    Transgenic mice overexpressing mutated NEBL, encoding the cardiac-specific Z-disk protein nebulette, develop severe cardiac phenotypes...
  14. doi Short-term use of telmisartan attenuates oxidation and improves Prdx2 expression more than antioxidant β-blockers in the cardiovascular systems of spontaneously hypertensive rats
    Sae Mi Yoo
    1 Laboratory of Cardiovascular Regeneration, Division of Cardiovascular Medicine, Seoul St Mary s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea 2 Department of Biomedical Science, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
    Hypertens Res 38:106-15. 2015
    ..In conclusion, TERT may positively modulate the balance between oxidative stress and antioxidant properties and demonstrate capabilities beyond its BP-lowering effects. ..
  15. doi Functional characterisation of different MLL fusion proteins by using inducible Sleeping Beauty vectors
    K Wachter
    Institute of Pharm Biology DCAL, Goethe University, Frankfurt Main, Germany
    Cancer Lett 352:196-202. 2014
    ..From these pilot experiments, we conclude that a systematic analysis of more MLL fusions will result in a more differentiated picture about the oncogenic capacity of distinct MLL fusions...
  16. doi A genome wide association study (GWAS) providing evidence of an association between common genetic variants and late radiotherapy toxicity
    Gillian C Barnett
    Centre for Cancer Genetic Epidemiology, University of Cambridge, Strangeways Research Laboratory, UK University of Cambridge, Department of Oncology, Oncology Centre, Cambridge University Hospitals NHS Foundation Trust, UK Electronic address
    Radiother Oncol 111:178-85. 2014
    ..This study was designed to identify common single nucleotide polymorphisms (SNPs) associated with toxicity 2years after radiotherapy...
  17. doi CUBN and NEBL common variants in the chromosome 10p13 linkage region are associated with multibacillary leprosy in Vietnam
    Audrey V Grant
    Laboratoire de Genetique Humaine des Maladies Infectieuses, Branche Necker, Institut National de la Sante et de la Recherche Medicale, U980, Paris, France
    Hum Genet 133:883-93. 2014
    ..Replicated significant association signals were revealed in the genes for cubilin (CUBN) and nebulette (NEBL). In the combined sample, the C allele (frequency 0...
  18. pmc Identification of Xin-repeat proteins as novel ligands of the SH3 domains of nebulin and nebulette and analysis of their interaction during myofibril formation and remodeling
    Stefan Eulitz
    Institute for Cell Biology, University of Bonn, D 53121 Bonn, Germany European Molecular Biology Laboratory Hamburg Deutsches Elektronen Synchrotron, D 22603 Hamburg, Germany Department of Medicinal Immunology, Charite University Medicine Berlin, D 13353 Berlin, Germany Department of Neurology, Neuromuscular Center Ruhrgebiet, University Hospital Bergmannsheil, Ruhr University Bochum, D 44789 Bochum, Germany
    Mol Biol Cell 24:3215-26. 2013
    ..We identify the SH3 domains of nebulin and nebulette as novel ligands of proline-rich regions of Xin and XIRP2...
  19. ncbi EG-1 interacts with c-Src and activates its signaling pathway
    Ming Lu
    Department of Surgery, Division of Oncology, UCLA School of Medicine, Los Angeles, CA, USA
    Int J Oncol 29:1013-8. 2006
    ..Furthermore, EG-1 shows interaction with multiple other SH3- and WW-containing molecules involved in various signaling pathways. These observations suggest that EG-1 may be involved in signaling pathways including c-Src activation...
  20. doi A newly characterized human well-differentiated liposarcoma cell line contains amplifications of the 12q12-21 and 10p11-14 regions
    Florence Pedeutour
    Institute for Research on Cancer and Aging, University of Nice Sophia Antipolis, Nice, France
    Virchows Arch 461:67-78. 2012
    ..The 10p amplicon contained 62 genes, including oncogenes such as MLLT10, previously described in chimeric fusion with MLL in leukemias, NEBL, and BMI1...
  21. doi Functional analysis of the two reciprocal fusion genes MLL-NEBL and NEBL-MLL reveal their oncogenic potential
    Mariana Emerenciano
    Pediatric Hematology Oncology Program, Research Center, Instituto Nacional de Cancer, Rio de Janeiro, Brazil
    Cancer Lett 332:30-4. 2013
    ..The results obtained in this study suggest that the reciprocal fusion genes of the Nebulin gene family might be of biological importance...
  22. pmc Investigating lasp-2 in cell adhesion: new binding partners and roles in motility
    Katherine T Bliss
    Department of Cellular and Molecular Medicine, University of Arizona, Tucson, AZ 85724, USA
    Mol Biol Cell 24:995-1006. 2013
    ..Lasp-2 (LIM-nebulette), a member of the nebulin family of actin-binding proteins, is a newly identified component of these complexes...
  23. pmc Altered regional cardiac wall mechanics are associated with differential cardiomyocyte calcium handling due to nebulette mutations in preclinical inherited dilated cardiomyopathy
    K Maiellaro-Rafferty
    The Heart Institute, Department of Pediatrics, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    J Mol Cell Cardiol 60:151-60. 2013
    b>Nebulette (NEBL) is a sarcomeric Z-disk protein involved in mechanosensing and force generation via its interaction with actin and tropomyosin-troponin complex...
  24. doi Mutational landscape of basal cell carcinomas by whole-exome sequencing
    Shyam S Jayaraman
    Division of Dermatology, Carilion Clinic and Virginia Tech Carilion School of Medicine, Roanoke, Virginia, USA
    J Invest Dermatol 134:213-20. 2014
    ..Our findings support the central role of PTCH1 mutations in BCC genesis. Moreover, our discovery of the uniquely high number of mutations in this tumor may lend insight into its biological behavior...
  25. ncbi Interstitial deletion of 10p and atrial septal defect in DiGeorge 2 syndrome
    S A Yatsenko
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA
    Clin Genet 66:128-36. 2004
    ..Considering that septal defects are the most frequent congenital heart anomaly, we suggest that further investigations in the 10p critical region are important to identify gene(s) responsible for this common birth defect...
  26. ncbi Characterization of the human nebulette gene: a polymorphism in an actin-binding motif is associated with nonfamilial idiopathic dilated cardiomyopathy
    T Arimura
    Department of Molecular Pathogenesis, Medical Research Institute, Tokyo Medical and Dental University, Japan
    Hum Genet 107:440-51. 2000
    ..related to Z-disc function have recently been reported to cause IDC, genomic organization of the gene for nebulette, a novel actin-binding Z-disc protein, was determined and its sequence variations were searched for in Japanese ..
  27. doi 2-D PAGE-based comparison of proteasome inhibitor bortezomib in sensitive and resistant mantle cell lymphoma
    Marc Weinkauf
    CCG Leukemia, Department of Medicine III, University Hospital Grosshadern LMU, Munich, Germany, in association with Helmholtz Center Munich, Munich, Germany
    Electrophoresis 30:974-86. 2009
    ..The results were confirmed in primary mantle cell lymphoma, thus confirming the critical role of these candidate proteins of proteasome inhibition...
  28. ncbi Genetic and comparative mapping of genes dysregulated in mouse hearts lacking the Hand2 transcription factor gene
    Melissa P Villanueva
    Department of Physiology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Genomics 80:593-600. 2002
    ..RACE analysis extended the sequence and identified this cDNA as the mouse ortholog of human nebulette, an actin-binding protein expressed in fetal heart...
  29. ncbi F-actin and myosin II binding domains in supervillin
    Yu Chen
    Department of Cell Biology, University of Massachusetts Medical School, Worcester, Massachusetts 01605, USA
    J Biol Chem 278:46094-106. 2003
    ..Taken together, these results suggest that supervillin may mediate actin and myosin II filament organization at cholesterol-rich membrane domains...
  30. pmc Plasmodium falciparum Pf34, a novel GPI-anchored rhoptry protein found in detergent-resistant microdomains
    Nicholas I Proellocks
    NHMRC Program in Malaria, Department of Microbiology, Monash University, VIC 3800, Australia
    Int J Parasitol 37:1233-41. 2007
    ..Pf34 is exposed to the human immune system during infection and is recognised by human immune sera collected from residents of malaria endemic areas of Vietnam and Papua New Guinea...
  31. ncbi Nebulette interacts with filamin C
    William B Holmes
    Department of Molecular and Cellular Biochemistry, University of Kentucky College of Medicine, Lexington, Kentucky 40536, USA
    Cell Motil Cytoskeleton 65:130-42. 2008
    The actin-binding proteins, nebulette, and nebulin, are comprised of a four-domain layout containing an acidic N-terminal region, a repeat domain, a serine-rich-linker region, and a Src homology-3 domain...
  32. pmc The LIM and SH3 domain protein family: structural proteins or signal transducers or both?
    Thomas G P Grunewald
    Department of Pediatrics, Klinikum rechts der Isar, Technische Universitat Munchen, Pediatric Oncology Center, Kolner Platz 1, D 80804 Munich, Germany
    Mol Cancer 7:31. 2008
    ..Subsequently it proved to be a splice variant of the Nebulin gene and therefore was also termed LIM/nebulette. LASP-1 and -2 are highly conserved in their LIM, nebulin-like and SH3 domains but differ significantly at their ..
  33. doi The nebulette repeat domain is necessary for proper maintenance of tropomyosin with the cardiac sarcomere
    Jeremy R Bonzo
    Department of Molecular and Cellular Biochemistry, University of Kentucky, 741 S Limestone, Lexington, KY 40536, USA
    Exp Cell Res 314:3519-30. 2008
    b>Nebulette is a cardiac-specific isoform of the giant actin-binding protein nebulin. Nebulette, having a mass of approximately 100 kDa, is only predicted to extend 150 nm from the edge of the Z-lines...
  34. doi Nebulette is the second member of the nebulin family fused to the MLL gene in infant leukemia
    Virginia M Coser
    Hematology Oncology Pediatric Program, CPq Instituto Nacional de Câncer, Rio de Janeiro, RJ20231 050, Brazil
    Cancer Genet Cytogenet 198:151-4. 2010
    ..Therefore, the combination of cytogenetic and independent molecular techniques such as long-distance inverse polymerase chain reaction are indispensable for the rapid identification and characterization of rare MLL rearrangements...
  35. doi Newly identified cytoskeletal components are associated with dynamic changes of podocyte foot processes
    Jing Miao
    1Department of Pediatrics, Peking University First Hospital, Beijing 100034, China
    Nephrol Dial Transplant 24:3297-305. 2009
    ..Here, the involved podocyte cytoskeletal molecules were explored on different days in puromycin aminonucleoside nephropathy rats...