inclusion bodies

Summary

Summary: A generic term for any circumscribed mass of foreign (e.g., lead or viruses) or metabolically inactive materials (e.g., ceroid or Mallory bodies), within the cytoplasm or nucleus of a cell. Inclusion bodies are in cells infected with certain filtrable viruses, observed especially in nerve, epithelial, or endothelial cells. (Stedman, 25th ed)

Top Publications

  1. ncbi TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
    Tetsuaki Arai
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Setagaya Ku, Tokyo 156 8585, Japan
    Biochem Biophys Res Commun 351:602-11. 2006
  2. ncbi Protein aggregation and neurodegenerative disease
    Christopher A Ross
    Division of Neurobiology, Department of Psychiatry, Johns Hopkins University School of Medicine, Ross Research Building, Room 618, 720 Rutland Avenue, Baltimore, Maryland 21205, USA
    Nat Med 10:S10-7. 2004
  3. ncbi FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis
    Han Xiang Deng
    Division of Neuromuscular Medicine, Davee Department of Neurology and Clinical Neurosciences, Chicago, IL 60611, USA
    Ann Neurol 67:739-48. 2010
  4. ncbi Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice
    Taichi Hara
    Department of Bioregulation and Metabolism, Tokyo Metropolitan Institute of Medical Science, Tokyo 113 8613, Japan
    Nature 441:885-9. 2006
  5. pmc Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity
    Yong Jie Zhang
    Department of Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Proc Natl Acad Sci U S A 106:7607-12. 2009
  6. ncbi Aggresomes, inclusion bodies and protein aggregation
    R R Kopito
    Dept of Biological Sciences, Stanford University, Stanford, CA 94305 5020, USA
    Trends Cell Biol 10:524-30. 2000
  7. pmc Chlamydia trachomatis co-opts GBF1 and CERT to acquire host sphingomyelin for distinct roles during intracellular development
    Cherilyn A Elwell
    Department of Medicine, University of California, San Francisco, California, United States of America
    PLoS Pathog 7:e1002198. 2011
  8. ncbi Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation
    Eric J Bennett
    Department of Biological Sciences, Stanford University, Stanford, CA 94305, USA
    Mol Cell 17:351-65. 2005
  9. pmc Inclusion bodies: a new concept
    Elena García-Fruitós
    CIBER en Bioingeniería, Biomateriales y Nanomedicina, Bellaterra, 08193 Barcelona, Spain
    Microb Cell Fact 9:80. 2010
  10. ncbi Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
    Montserrat Arrasate
    Gladstone Institute of Neurological Disease, University of California, San Francisco, California 94141, USA
    Nature 431:805-10. 2004

Detail Information

Publications341 found, 100 shown here

  1. ncbi TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
    Tetsuaki Arai
    Department of Psychogeriatrics, Tokyo Institute of Psychiatry, Setagaya Ku, Tokyo 156 8585, Japan
    Biochem Biophys Res Commun 351:602-11. 2006
    ....
  2. ncbi Protein aggregation and neurodegenerative disease
    Christopher A Ross
    Division of Neurobiology, Department of Psychiatry, Johns Hopkins University School of Medicine, Ross Research Building, Room 618, 720 Rutland Avenue, Baltimore, Maryland 21205, USA
    Nat Med 10:S10-7. 2004
    ..There is now increased understanding of the pathways involved in protein aggregation, and some recent clues have emerged as to the molecular mechanisms of cellular toxicity. These are leading to approaches toward rational therapeutics...
  3. ncbi FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis
    Han Xiang Deng
    Division of Neuromuscular Medicine, Davee Department of Neurology and Clinical Neurosciences, Chicago, IL 60611, USA
    Ann Neurol 67:739-48. 2010
    ..The pathogenic mechanism of the mutant FUS-mediated ALS and potential roles of FUS in non-FUS ALS remain to be investigated...
  4. ncbi Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice
    Taichi Hara
    Department of Bioregulation and Metabolism, Tokyo Metropolitan Institute of Medical Science, Tokyo 113 8613, Japan
    Nature 441:885-9. 2006
    ..cells develop progressive deficits in motor function that are accompanied by the accumulation of cytoplasmic inclusion bodies in neurons...
  5. pmc Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity
    Yong Jie Zhang
    Department of Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Proc Natl Acad Sci U S A 106:7607-12. 2009
    ..Finally, by generating a conformation-dependent antibody that detects C-terminal fragments, we show that this toxic cleavage product is specific for pathologic inclusions in human TDP-43 proteinopathies...
  6. ncbi Aggresomes, inclusion bodies and protein aggregation
    R R Kopito
    Dept of Biological Sciences, Stanford University, Stanford, CA 94305 5020, USA
    Trends Cell Biol 10:524-30. 2000
    ..Once formed, aggregates tend to be refractory to proteolysis and to accumulate in inclusion bodies. This accumulation has been assumed to be a diffusion-limited process, but recent studies suggest that, in ..
  7. pmc Chlamydia trachomatis co-opts GBF1 and CERT to acquire host sphingomyelin for distinct roles during intracellular development
    Cherilyn A Elwell
    Department of Medicine, University of California, San Francisco, California, United States of America
    PLoS Pathog 7:e1002198. 2011
    ..Our results reveal a novel mechanism by which an intracellular pathogen redirects SM biosynthesis to its replicative niche...
  8. ncbi Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation
    Eric J Bennett
    Department of Biological Sciences, Stanford University, Stanford, CA 94305, USA
    Mol Cell 17:351-65. 2005
    ..shown that the UPS is impaired in the presence of aggregated proteins that become deposited into cytoplasmic inclusion bodies (IBs)...
  9. pmc Inclusion bodies: a new concept
    Elena García-Fruitós
    CIBER en Bioingeniería, Biomateriales y Nanomedicina, Bellaterra, 08193 Barcelona, Spain
    Microb Cell Fact 9:80. 2010
    ..Therefore, the development of new purification protocols aimed to preserve those properties is becoming a pushing demand...
  10. ncbi Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
    Montserrat Arrasate
    Gladstone Institute of Neurological Disease, University of California, San Francisco, California 94141, USA
    Nature 431:805-10. 2004
    ..by an abnormal polyglutamine expansion within the protein huntingtin and is characterized by microscopic inclusion bodies of aggregated huntingtin and by the death of selected types of neuron...
  11. pmc Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis
    Jason Miller
    Gladstone Institute of Neurological Disease, University of California, San Francisco, California 94158, USA
    J Neurosci 30:10541-50. 2010
    An expanded polyglutamine (polyQ) stretch in the protein huntingtin (htt) induces self-aggregation into inclusion bodies (IBs) and causes Huntington's disease (HD)...
  12. pmc Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
    Masato Hasegawa
    Department of Molecular Neurobiology, Tokyo Institute of Psychiatry, Tokyo Metropolitan Organization for Medical Research, Kamikitazawa, Setagaya Ku, Tokyo, Japan
    Ann Neurol 64:60-70. 2008
    ..The aim of this study was to identify the phosphorylation sites and responsible kinases, and to clarify the pathological significance of phosphorylation of TDP-43...
  13. pmc Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates
    Pei Hsien Ren
    Department of Biology, Stanford University, Stanford, CA 94305 5020, USA
    Nat Cell Biol 11:219-25. 2009
    ....
  14. ncbi Toxic proteins in neurodegenerative disease
    J Paul Taylor
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Bethesda, MD 20892, USA
    Science 296:1991-5. 2002
    ..Increased understanding of the cellular mechanisms for disposal of abnormal proteins and of the effects of toxic protein accumulation on neuronal survival may allow the development of rational, effective treatment for these disorders...
  15. ncbi Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS
    Daisuke Ito
    Department of Neurology, School of Medicine, Keio University, Tokyo, Japan
    Ann Neurol 69:152-62. 2011
    ..identified as a cause of familial amyotrophic lateral sclerosis (ALS), as well as a major component of the inclusion bodies found in subtypes of frontotemporal lobar degeneration (FTLD)...
  16. pmc Mechanisms of host cell exit by the intracellular bacterium Chlamydia
    Kevin Hybiske
    Division of Infectious Diseases, School of Public Health, University of California, Berkeley, CA 94720, USA
    Proc Natl Acad Sci U S A 104:11430-5. 2007
    ..The participation of Rho was unique in that it functioned late in extrusion. The dual nature of release characterized for Chlamydia has not been observed as a strategy for intracellular bacteria...
  17. pmc p62 Is a common component of cytoplasmic inclusions in protein aggregation diseases
    Kurt Zatloukal
    Department of Pathology, Karl Franzens University, Graz, Austria
    Am J Pathol 160:255-63. 2002
    ..The presence of p62 along with other stress proteins and ubiquitin in cytoplasmic inclusions indicates deposition as aggregates as a third line of defense against misfolded proteins in addition to refolding and degradation...
  18. ncbi Loss of autophagy in the central nervous system causes neurodegeneration in mice
    Masaaki Komatsu
    Laboratory of Frontier Science, Tokyo Metropolitan Institute of Medical Science, Bunkyo ku, Tokyo 113 8613, Japan
    Nature 441:880-4. 2006
    ..Notably, polyubiquitinated proteins accumulated in autophagy-deficient neurons as inclusion bodies, which increased in size and number with ageing...
  19. pmc Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U
    Kuen Jer Tsai
    Institute of Clinical Medicine, National Cheng Kung University, Tainan 704, Taiwan
    J Exp Med 207:1661-73. 2010
    ..This FTLD-U mouse model should be valuable for the mechanistic analysis of the role of TDP-43 in the pathogenesis of FTLD-U and for the design of effective therapeutic approaches of the disease...
  20. pmc SNARE protein mimicry by an intracellular bacterium
    Cedric Delevoye
    Institut Pasteur, Unite de Biologie des Interactions Cellulaires, CNRS URA 2582, Paris, France
    PLoS Pathog 4:e1000022. 2008
    ..Moreover, point mutations in the central layer of IncA SNARE-like motifs resulted in the loss of binding to host SNAREs. Altogether, our data demonstrate for the first time mimicry of the SNARE motif by a bacterium...
  21. ncbi Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice
    V C Wheeler
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Hum Mol Genet 9:503-13. 2000
    ....
  22. ncbi Protein aggregation and amyloid fibril formation prediction software from primary sequence: towards controlling the formation of bacterial inclusion bodies
    Stavros J Hamodrakas
    Department of Cell Biology and Biophysics, Faculty of Biology, University of Athens, Athens, Greece
    FEBS J 278:2428-35. 2011
    ..One such example is given...
  23. pmc Novel cell- and tissue-based assays for detecting misfolded and aggregated protein accumulation within aggresomes and inclusion bodies
    Dee Shen
    Enzo Life Sciences, 10 Executive Blvd, Farmingdale, NY 11735, USA
    Cell Biochem Biophys 60:173-85. 2011
    Aggresomes and related inclusion bodies appear to serve as storage depots for misfolded and aggregated proteins within cells, which can potentially be degraded by the autophagy pathway...
  24. pmc The hypothetical protein CT813 is localized in the Chlamydia trachomatis inclusion membrane and is immunogenic in women urogenitally infected with C. trachomatis
    Chaoqun Chen
    Department of Microbiology and Immunology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, Texas 78229, USA
    Infect Immun 74:4826-40. 2006
    ..Thus, the CT813 protein may represent an important candidate for understanding C. trachomatis pathogenesis and developing intervention and prevention strategies for controlling C. trachomatis infection...
  25. ncbi Impairment of the ubiquitin-proteasome system by protein aggregation
    N F Bence
    Department of Biological Sciences, Stanford University, Stanford, CA 94305-5020, USA
    Science 292:1552-5. 2001
    ....
  26. ncbi Neurological abnormalities in a knock-in mouse model of Huntington's disease
    C H Lin
    Department of Biochemistry and Molecular Genetics, University of Alabama at Birmingham, Birmingham, AL 35294, USA
    Hum Mol Genet 10:137-44. 2001
    ..These mice will serve as useful reagents in experiments designed to reveal the molecular nature of neuronal dysfunction underlying HD...
  27. pmc TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration
    Hans Wils
    Department of Molecular Genetics, VIB, B 2610 Antwerpen, Belgium
    Proc Natl Acad Sci U S A 107:3858-63. 2010
    ..These findings suggest that approximately 25-kDa TDP-43 CTFs are noxious to neurons by a gain of aberrant nuclear function...
  28. ncbi Inclusion bodies: specificity in their aggregation process and amyloid-like structure
    Montse Morell
    Institut de Biotecnologia i de Biomedicina, Departament de Bioquimica i Biologia Molecular, Universitat Autonoma de Barcelona, Spain
    Biochim Biophys Acta 1783:1815-25. 2008
    ..Overall, our results suggest that conserved mechanisms underlie protein aggregation in different organisms. They also have important implications for biotechnological and biomedical applications of recombinant polypeptides...
  29. pmc A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity
    Brian S Johnson
    Department of Cell and Developmental Biology, University of Pennsylvania, Philadelphia, PA 19104, USA
    Proc Natl Acad Sci U S A 105:6439-44. 2008
    ..This work provides a mechanistic framework for investigating the toxicity of TDP-43 aggregation relevant to human disease and establishes a manipulable, high-throughput model for discovering potential therapeutic strategies...
  30. ncbi Bacterial inclusion bodies: making gold from waste
    Elena García-Fruitós
    Institute for Biotechnology and Biomedicine, Universitat Autonoma de Barcelona, Bellaterra, 08193 Barcelona, Spain
    Trends Biotechnol 30:65-70. 2012
    Many protein species produced in recombinant bacteria aggregate as insoluble protein clusters named inclusion bodies (IBs). IBs are discarded from further processing or are eventually used as a pure protein source for in vitro refolding...
  31. pmc Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion
    Johannes Brettschneider
    Center for Neurodegenerative Disease Research CNDR, University of Pennsylvania School of Medicine, 3rd Floor Maloney Building, 3600 Spruce Street, Philadelphia, PA 19104, USA
    Acta Neuropathol 123:825-39. 2012
    ..Our study indicates that this pathology is associated with alterations in clinical phenotype, and suggests that the presence of C9ORF72 repeat expansions may indicate a worse prognosis in ALS...
  32. pmc Impairment of starvation-induced and constitutive autophagy in Atg7-deficient mice
    Masaaki Komatsu
    Department of Molecular Oncology, Tokyo Metropolitan Institute of Medical Science, Bunkyo ku, Tokyo 113 8613, Japan
    J Cell Biol 169:425-34. 2005
    ..Our results indicate the important role of autophagy in starvation response and the quality control of proteins and organelles in quiescent cells...
  33. pmc Nuclear localization sequence of FUS and induction of stress granules by ALS mutants
    Jozsef Gal
    Department of Molecular and Cellular Biochemistry, College of Medicine, University of Kentucky, Lexington, KY 40536, USA
    Neurobiol Aging 32:2323.e27-40. 2011
    ..Our results suggest that the ALS mutations in FUS NLS can impair FUS nuclear localization, induce cytoplasmic inclusions and stress granules, and potentially perturb RNA metabolism...
  34. pmc Cytoplasmic lipid droplets are translocated into the lumen of the Chlamydia trachomatis parasitophorous vacuole
    Jordan L Cocchiaro
    Center for Microbial Pathogenesis, Department of Molecular Genetics and Microbiology, Duke University Medical Center, Durham, NC 27710, USA
    Proc Natl Acad Sci U S A 105:9379-84. 2008
    ..The wholesale transport of LDs into the lumen of a parasitophorous vacuole represents a unique mechanism of organelle sequestration and subversion by a bacterial pathogen...
  35. ncbi Decreased proteolysis caused by protein aggregates, inclusion bodies, plaques, lipofuscin, ceroid, and 'aggresomes' during oxidative stress, aging, and disease
    Tilman Grune
    Research Institute of Environmental Medicine, Heinrich Heine University Dusseldorf, Auf m Hennekamp 50, 40225 Dusseldorf, Germany
    Int J Biochem Cell Biol 36:2519-30. 2004
    ..Such aggregates are also called inclusion bodies, plaques, lipofuscin, ceroid, or 'aggresomes' depending on their location and composition...
  36. ncbi Protein aggregation: folding aggregates, inclusion bodies and amyloid
    A L Fink
    Department of Chemistry and Biochemistry, University of California, Santa Cruz 95064, USA
    Fold Des 3:R9-23. 1998
    Aggregation results in the formation of inclusion bodies, amyloid fibrils and folding aggregates...
  37. ncbi Lysine 63-linked ubiquitination promotes the formation and autophagic clearance of protein inclusions associated with neurodegenerative diseases
    Jeanne M M Tan
    Neurodegeneration Research Lab, National Neuroscience Institute, Singapore, Singapore
    Hum Mol Genet 17:431-9. 2008
    ..Collectively, our results provide a novel mechanistic route that underlies the life cycle of an inclusion body. Harnessing this pathway may offer innovative approaches in the treatment of neurodegenerative disorders...
  38. ncbi Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
    S W Davies
    Department of Anatomy and Developmental Biology, University College London, United Kingdom
    Cell 90:537-48. 1997
    ..The appearance in transgenic mice of these inclusions, followed by characteristic morphological change within neuronal nuclei, is strikingly similar to nuclear abnormalities observed in biopsy material from HD patients...
  39. pmc Specific chlamydial inclusion membrane proteins associate with active Src family kinases in microdomains that interact with the host microtubule network
    Jeffrey Mital
    Host Parasite Interactions Section, Laboratory of Intracellular Parasites, National Institute of Allergy and Infectious Diseases, Rocky Mountain Laboratories, NIAID, NIH, Hamilton, MT 59840, USA
    Cell Microbiol 12:1235-49. 2010
    ..Together, the data suggest that a specific structure on the C. trachomatis inclusion membrane may be responsible for the known interactions of chlamydiae with the microtubule network and resultant effects on centrosome stability...
  40. ncbi Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic
    Toshiaki Takahashi
    School of Health Sciences, Faculty of Medicine, Niigata University, 1 757 Asahimachi, Niigata 951 8122, Japan
    Hum Mol Genet 17:345-56. 2008
    ..polyQ oligomers have been proposed as a cytotoxic structure, the cytotoxicity of soluble polyQ oligomers, not inclusion bodies (IBs), has not been proven in living cells...
  41. ncbi Amyloid-like properties of bacterial inclusion bodies
    Mar Carrió
    Institut de Biotecnologia i de Biomedicina, Universitat Autonoma de Barcelona, 08193 Bellaterra Barcelona, Spain
    J Mol Biol 347:1025-37. 2005
    Bacterial inclusion bodies are major bottlenecks in protein production, narrowing the spectrum of relevant polypeptides obtained by recombinant DNA...
  42. pmc Actin and intermediate filaments stabilize the Chlamydia trachomatis vacuole by forming dynamic structural scaffolds
    Yadunanda Kumar
    Department of Molecular Genetics and Microbiology and Center for Microbial Pathogenesis, Duke University Medical Center, Durham, NC 27710, USA
    Cell Host Microbe 4:159-69. 2008
    ..We propose that Chlamydia has co-opted the function of F-actin and IFs to stabilize the inclusion with a dynamic, structural scaffold while minimizing the exposure of inclusion contents to cytoplasmic innate immune-surveillance pathways...
  43. ncbi Functional inclusion bodies produced in bacteria as naturally occurring nanopills for advanced cell therapies
    Esther Vazquez
    Institut de Biotecnologia i de Biomedicina, Universitat Autonoma de Barcelona, Bellaterra, Spain
    Adv Mater 24:1742-7. 2012
    b>Inclusion bodies (50-500 nm in diameter) produced in recombinant bacteria can be engineered to contain functional proteins with therapeutic potential...
  44. ncbi Bacterial inclusion bodies of Alzheimer's disease β-amyloid peptides can be employed to study native-like aggregation intermediate states
    Muralidhar Dasari
    Leibniz Institut fur Molekulare Pharmakologie, Robert Rossle Strasse 10, 13125 BerlinBuch, Germany
    Chembiochem 12:407-23. 2011
    ..Bacterial inclusion bodies contain large amounts of small heat shock proteins (sHSPs), which are highly homologous to those found in the ..
  45. ncbi Progressive motor weakness in transgenic mice expressing human TDP-43
    Nancy R Stallings
    Department of Neurology, University of Texas, Southwestern Medical Center, Dallas, TX 75390, USA
    Neurobiol Dis 40:404-14. 2010
    ..Transgenic mouse lines expressing untagged mutant and wild type TDP-43 under the same promoter represent a powerful new model system for studying TDP-43 proteinopathies in vivo...
  46. pmc Packaging protein drugs as bacterial inclusion bodies for therapeutic applications
    Antonio Villaverde
    Institut de Biotecnologia i de Biomedicina, Universitat Autonoma de Barcelona, Bellaterra 08193, Barcelona, Spain
    Microb Cell Fact 11:76. 2012
    A growing number of insights on the biology of bacterial inclusion bodies (IBs) have revealed intriguing utilities of these protein particles...
  47. ncbi Biological role of bacterial inclusion bodies: a model for amyloid aggregation
    Elena García-Fruitós
    Institute for Biotechnology and Biomedicine, Universitat Autonoma de Barcelona, Barcelona, Spain
    FEBS J 278:2419-27. 2011
    b>Inclusion bodies are insoluble protein aggregates usually found in recombinant bacteria when they are forced to produce heterologous protein species...
  48. ncbi Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein
    J Paul Taylor
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, Building 10, Room 3B 14, Bethesda, MD 20892 1250, USA
    Hum Mol Genet 12:749-57. 2003
    ..Together, these findings suggest that aggresomes are cytoprotective, serving as cytoplasmic recruitment centers to facilitate degradation of toxic proteins...
  49. ncbi Colocalization of transactivation-responsive DNA-binding protein 43 and huntingtin in inclusions of Huntington disease
    Claudia Schwab
    Kinsmen Laboratory of Neurological Research, University of British Columbia, Vancouver, British Columbia, Canada
    J Neuropathol Exp Neurol 67:1159-65. 2008
    ..Our results further add to the hypothesis that TDP-43 may be involved in the pathology of a variety of neurodegenerative disorders...
  50. pmc Isolates of Chlamydia trachomatis that occupy nonfusogenic inclusions lack IncA, a protein localized to the inclusion membrane
    R J Suchland
    Division of Allergy and Infectious Diseases, School of Medicine, University of Washington, Seattle, Washington, USA
    Infect Immun 68:360-7. 2000
    ..trachomatis isolates that form nonfusing inclusions; the variant phenotype is associated with the absence of detectable IncA and with an altered incA sequence that modifies the characteristic hydrophobic domain of the IncA protein...
  51. ncbi Lewy-body formation is an aggresome-related process: a hypothesis
    C Warren Olanow
    Department of Neurology, Mount Sinai School of Medicine, New York, NY 10029, USA
    Lancet Neurol 3:496-503. 2004
    ..The concept of Lewy bodies as aggresome-related inclusions fits well with ongoing discoveries suggesting that altered protein handling might contribute to the neurodegenerative process in familial and sporadic forms of PD...
  52. ncbi The cellular fate of mutant rhodopsin: quality control, degradation and aggresome formation
    Richard S Saliba
    Division of Pathology, Institute of Ophthalmology, University College London, UK
    J Cell Sci 115:2907-18. 2002
    ....
  53. pmc Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases
    Ruth A Bodner
    Center for Cancer Research, Massachusetts Institute of Technology, Room E18 505, 77 Massachusetts Avenue, Cambridge, MA 02139, USA
    Proc Natl Acad Sci U S A 103:4246-51. 2006
    ....
  54. ncbi FUS pathology in basophilic inclusion body disease
    David G Munoz
    Division of Pathology, Department of Laboratory Medicine and Pathobiology, Room 2 097 Cardinal Carter, St Michael s Hospital, University of Toronto, 30 Bond Street, Toronto, ON M5B 1W8, Canada
    Acta Neuropathol 118:617-27. 2009
    ..The consistent involvement of motorneurons in BIBD indicates that the association of FTLD and MND/ALS can occur on a FUS or TDP-43 pathological substrate...
  55. ncbi Amyloids in bacterial inclusion bodies
    Natalia S de Groot
    Departament de Bioquímica i Biologia Molecular and Institut de Biotecnologia i de Biomedicina, Universitat Autonoma de Barcelona, 08193 Bellaterra, Barcelona, Spain
    Trends Biochem Sci 34:408-16. 2009
    ..provided compelling evidence for the existence of highly ordered, amyloid-like conformations in the insoluble inclusion bodies produced during heterologous protein expression in bacteria...
  56. ncbi Pathological inclusion bodies in tauopathies contain distinct complements of tau with three or four microtubule-binding repeat domains as demonstrated by new specific monoclonal antibodies
    R De Silva
    Reta Lila Weston Institute of Neurological Studies, University College London, and Department of Molecular Neuroscience and Neuropathology, Institute of Neurology, UK
    Neuropathol Appl Neurobiol 29:288-302. 2003
    ..These new isoform-specific antibodies are useful tools for analysing tau isoform expression and distribution as well as pathological changes in the human brain...
  57. ncbi From Mallory to Mallory-Denk bodies: what, how and why?
    Kurt Zatloukal
    Institute of Pathology, Medical University of Graz, Auenbruggerplatz 25, A 8036 Graz, Austria
    Exp Cell Res 313:2033-49. 2007
    ..Although it remains unclear whether MDBs serve a bystander, protective or injury promoting function, they do serve an important role as histological and potential progression markers in several liver diseases...
  58. pmc Restricted fusion of Chlamydia trachomatis vesicles with endocytic compartments during the initial stages of infection
    Marci A Scidmore
    Host Parasite Interactions Section, Laboratory of Intracellular Parasites, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, Hamilton, Montana 59840, USA
    Infect Immun 71:973-84. 2003
    ....
  59. ncbi Mammalian 14-3-3beta associates with the Chlamydia trachomatis inclusion membrane via its interaction with IncG
    M A Scidmore
    Host Parasite Interactions Section, Laboratory of Intracellular Parasites, National Institutes of Allergy and Infectious Diseases, Rocky Mountain Laboratories, Hamilton, MT 59840, USA
    Mol Microbiol 39:1638-50. 2001
    ..psittaci or C. pneumoniae inclusions. 14-3-3beta is the first eukaryotic protein found to interact with the chlamydial inclusion; however, its unique role in C. trachomatis pathogenesis remains to be determined...
  60. ncbi Chaperone suppression of alpha-synuclein toxicity in a Drosophila model for Parkinson's disease
    Pavan K Auluck
    Department of Neuroscience, University of Pennsylvania and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Science 295:865-8. 2002
    ..Furthermore, Lewy bodies in human postmortem tissue immunostained for molecular chaperones, also suggesting that chaperones may play a role in Parkinson's disease progression...
  61. ncbi Global changes to the ubiquitin system in Huntington's disease
    Eric J Bennett
    Department of Biological Sciences, Stanford University, Stanford, California 94305, USA
    Nature 448:704-8. 2007
    ..Abnormal enrichment of HD inclusion bodies with ubiquitin, a diagnostic characteristic of HD and many other neurodegenerative disorders including ..
  62. pmc High-throughput automated refolding screening of inclusion bodies
    Renaud Vincentelli
    Architecture et Fonction des Macromolecules Biologiques, UMR 6098, CNRS et Universités d Aix Marseille I et II, 31 Chemin Joseph Aiguier, 13402 Marseille Cedex 20, France
    Protein Sci 13:2782-92. 2004
    ..foreign proteins in Escherichia coli is the occurrence of amorphous aggregates of misfolded proteins, called inclusion bodies (IB)...
  63. ncbi Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice
    Liliana B Menalled
    Department of Neurology, University of California Los Angeles School of Medicine, Los Angeles, California 90095, USA
    J Neurosci 22:8266-76. 2002
    ....
  64. pmc The chlamydial plasmid-encoded protein pgp3 is secreted into the cytosol of Chlamydia-infected cells
    Zhongyu Li
    Department of Microbiology and Immunology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78229, USA
    Infect Immun 76:3415-28. 2008
    ....
  65. pmc Bacterial inclusion bodies contain amyloid-like structure
    Lei Wang
    Structural Biology Laboratory, The Salk Institute, La Jolla, California, United States of America
    PLoS Biol 6:e195. 2008
    ..We studied the structure of bacterial inclusion bodies, which have been believed to belong to the amorphous class of aggregates...
  66. pmc Temporal analysis of the developing Chlamydia psittaci inclusion by use of fluorescence and electron microscopy
    D D Rockey
    Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, Hamilton, Montana 59840, USA
    Infect Immun 64:4269-78. 1996
    ..The division of inclusions slows or stops in mid-cycle, and dividing RB accumulate within the enlarging lobes. The RB then differentiate to EBs, the inclusion and cell are lysed, and EBs are freed to infect another cell...
  67. pmc Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions
    Eric J Huang
    Department of Pathology, University of California, San Francisco, CA, USA
    Brain Pathol 20:1069-76. 2010
    ..Furthermore, our study represents the first detailed characterizations of neuropathological findings in rapidly progressive juvenile ALS patients with a mutation in the FUS/TLS gene...
  68. ncbi Mouse model of multiple system atrophy alpha-synuclein expression in oligodendrocytes causes glial and neuronal degeneration
    Ikuru Yazawa
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Neuron 45:847-59. 2005
    ....
  69. pmc Ultrastructural localization of TDP-43 in filamentous neuronal inclusions in various neurodegenerative diseases
    Wen Lang Lin
    Department of Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL, 32224, USA
    Acta Neuropathol 116:205-13. 2008
    ..The results suggest that TDP-43 is the primary component of the granulofilamentous inclusions in FTLD-U and ALS. Similar inclusions sometimes accompany filamentous aggregates composed of other abnormal proteins in AD, PiD and LBD...
  70. ncbi Polyglutamine protein aggregates are dynamic
    Soojin Kim
    Department of Biochemistry, Molecular Biology and Cell Biology, Rice Institute for Biomedical Research, Northwestern University, Evanston, IL 60208, USA
    Nat Cell Biol 4:826-31. 2002
    Protein aggregation and the formation of inclusion bodies are hallmarks of the cytopathology of neurodegenerative diseases, including Huntington's disease, Amyotropic lateral sclerosis, Parkinson's disease and Alzheimer's disease...
  71. ncbi The Mallory body: morphological, clinical and experimental studies (Part 1 of a literature survey)
    K Jensen
    Department of Medical Gastroenterology, Hvidovre University Hospital, Denmark
    Hepatology 20:1061-77. 1994
    ..In conclusion, the Mallory body is indicative but not pathognomonic of alcohol involvement. A discussion on theories of development and pathological significance transcending the clinical frameworks will be presented in a future paper...
  72. ncbi Expression, purification, and refolding of biologically active Acinetobacter baumannii OmpA from Escherichia coli inclusion bodies
    Michael J McConnell
    Unit of Infectious Disease, Microbiology, and Preventive Medicine, Institute of Biomedicine of Sevilla, University Hospital Virgen del Rocío CSIC University of Sevilla, 41013 Sevilla, Spain
    Protein Expr Purif 77:98-103. 2011
    ..OmpA was cloned into the vector pET-15b and purified under denaturing conditions from Escherichia coli inclusion bodies using nickel affinity chromatography...
  73. ncbi Co-morbidity of TDP-43 proteinopathy in Lewy body related diseases
    Hanae Nakashima-Yasuda
    Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, HUP Maloney 3rd Floor, Philadelphia, PA 19104 4283, USA
    Acta Neuropathol 114:221-9. 2007
    ..This study expands the concept of TDP-43 proteinopathies by implicating TDP-43 lesions in mechanisms of neurodegeneration in LB disorders...
  74. ncbi Recombinant protein folding and misfolding in Escherichia coli
    Francois Baneyx
    Departments of Chemical Engineering and Bioengineering, University of Washington, Box 351750, Seattle, Washington 98195, USA
    Nat Biotechnol 22:1399-408. 2004
    ..coli will remain an important host for the production of both commodity and high value added proteins...
  75. ncbi Searching for the cause of Kawasaki disease--cytoplasmic inclusion bodies provide new insight
    Anne H Rowley
    Department of Pediatrics, Northwestern University Feinberg School of Medicine, The Center for Kawasaki Disease, The Children s Memorial Hospital, Chicago, Illinois 60611, USA
    Nat Rev Microbiol 6:394-401. 2008
    ..Recently, identification of an antigen-driven IgA response that was directed at cytoplasmic inclusion bodies in KD tissues has provided new insights that could unlock the mysteries of KD.
  76. pmc Toll-like receptor 2 activation by Chlamydia trachomatis is plasmid dependent, and plasmid-responsive chromosomal loci are coordinately regulated in response to glucose limitation by C. trachomatis but not by C. muridarum
    CATHERINE M O'CONNELL
    Department of Pediatrics, Children s Hospital of Pittsburgh of UPMC, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA
    Infect Immun 79:1044-56. 2011
    ..Glucose-limited C. trachomatis displayed reduced TLR2 stimulation in an in vitro assay. During human chlamydial infection, glucose limitation may decrease chlamydial virulence through its effects on plasmid-responsive chromosomal genes...
  77. ncbi p62/sequestosome 1 binds to TDP-43 in brains with frontotemporal lobar degeneration with TDP-43 inclusions
    Kunikazu Tanji
    Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki, Japan
    J Neurosci Res 90:2034-42. 2012
    ..Our results suggest that the interaction of TDP-43 and p62 is disrupted and may participate in the pathogenesis of TDP-43 proteinopathy...
  78. pmc RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations
    J Gavin Daigle
    Department of Genetics, Louisiana State University Health Sciences Center, New Orleans, LA, USA
    Hum Mol Genet 22:1193-205. 2013
    ..In summary, we demonstrate that the RNA-binding ability of FUS is essential for the neurodegenerative phenotype in vivo of mutant FUS (either through direct contact with RNA or through interactions with other RBPs)...
  79. pmc The E3-ubiquitin ligase TRIM50 interacts with HDAC6 and p62, and promotes the sequestration and clearance of ubiquitinated proteins into the aggresome
    Carmela Fusco
    Medical Genetics Unit, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Italy
    PLoS ONE 7:e40440. 2012
    ..We speculate that when the proteasome activity is impaired, TRIM50 fails to drive its substrates to the proteasome-mediated degradation, and promotes their storage in the aggresome for successive clearance...
  80. ncbi Temperature effect on inclusion body formation and stress response in the periplasm of Escherichia coli
    Sabine Hunke
    Humboldt Universitat zu Berlin, Institut für Biologie Bakterienphysiologie, Chausseestr 117, D 10115 Berlin, Germany
    Mol Microbiol 50:1579-89. 2003
    ..a defective-folding mutant of maltose-binding protein of Escherichia coli, MalE31, which formed periplasmic inclusion bodies. Here, we show that MalE31 aggregation does not affect bacterial growth at 30 degrees C but is lethal at 37 ..
  81. ncbi Chromatoid bodies: aggresome-like characteristics and degradation sites for organelles of spermiogenic cells
    Celina M Haraguchi
    Biology Laboratory, University of Yamanashi, Yamanashi, Japan
    J Histochem Cytochem 53:455-65. 2005
    ..These results show that the CBs have some aggresomal features, suggesting that they are not a synthetic site as proposed previously but a degradation site where unnecessary DNA, RNA, and proteins are digested...
  82. pmc Chlamydia trachomatis intercepts Golgi-derived sphingolipids through a Rab14-mediated transport required for bacterial development and replication
    Anahí Capmany
    Cell Biology Laboratory, Biochemistry Department, IHEM CONICET, Faculty of Medicine, University of Cuyo, Mendoza, Argentina
    PLoS ONE 5:e14084. 2010
    ..Novel anti-chlamydial therapies could be developed based on the knowledge of how bacteria subvert host vesicular transport events through Rabs manipulation...
  83. pmc Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43)
    Eva Bentmann
    Adolf Butenandt Institute, Biochemistry, Ludwig Maximilians University, Munchen, Germany
    J Biol Chem 287:23079-94. 2012
    ....
  84. pmc Inclusion bodies as potential vehicles for recombinant protein delivery into epithelial cells
    Mirjana Liovic
    Medical Center for Molecular Biology, University of Ljubljana, Ljubljana, Slovenia
    Microb Cell Fact 11:67. 2012
    We present the potential of inclusion bodies (IBs) as a protein delivery method for polymeric filamentous proteins. We used as cell factory a strain of E...
  85. pmc Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion
    Jacqueline C Mitchell
    Department of Clinical Neuroscience, Kings College London, King s Centre for Neurodegeneration Research, London SE5 8AF, UK
    Acta Neuropathol 125:273-88. 2013
    ..Furthermore, these mice will provide a new model to study disease mechanism, and test therapies...
  86. pmc Identifying polyglutamine protein species in situ that best predict neurodegeneration
    Jason Miller
    Gladstone Institute of Neurological Disease, San Francisco, California, USA
    Nat Chem Biol 7:925-34. 2011
    ..Although polyQ expansions render proteins prone to aggregate into inclusion bodies, this may be a neuronal coping response to more toxic forms of polyQ...
  87. pmc Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis
    Christophe Verbeeck
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA
    Mol Neurodegener 7:53. 2012
    ..In this paper we report the development of somatic brain transgenic (SBT) mice using recombinant adeno-associated virus (rAAV) to investigate how FUS mutations lead to neurodegeneration...
  88. pmc Using bacterial inclusion bodies to screen for amyloid aggregation inhibitors
    Anna Villar-Piqué
    Departament de Bioquimica i Biologia Molecular, Facultat de Biociencies, Universitat Autonoma de Barcelona, E 08193, Bellaterra, Spain
    Microb Cell Fact 11:55. 2012
    ..In particular, the presence of endogenous metal ions has been linked to the pathogenesis of AD and other neurodegenerative disorders...
  89. ncbi Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse
    J M Ordway
    Department of Biochemistry and Molecular Genetics, University of Alabama at Birmingham, 35294, USA
    Cell 91:753-63. 1997
    ..Neuronal intranuclear inclusions are present in affected mice. Our results show that CAG repeats do not need to be located within one of the classic repeat disorder genes to have a neurotoxic effect...
  90. pmc Small surfactant-like peptides can drive soluble proteins into active aggregates
    Bihong Zhou
    Department of Chemical Engineering, Tsinghua University, One Tsinghua Garden Road, Beijing 100084, China
    Microb Cell Fact 11:10. 2012
    Inactive protein inclusion bodies occur commonly in Escherichia coli (E. coli) cells expressing heterologous proteins...
  91. ncbi TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations
    Manuela Neumann
    Center for Neuropathology and Prion Research, Ludwig Maximilians University, Munich, Germany
    J Neuropathol Exp Neurol 66:152-7. 2007
    ..TDP-43 is a common pathologic substrate linking a variety of distinct patterns of FTLD-U pathology caused by different genetic alterations...
  92. ncbi Evidence of multisystem disorder in whole-brain map of pathological TDP-43 in amyotrophic lateral sclerosis
    Felix Geser
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Alzheimer s Disease Core Center, Institute on Aging, Philadelphia, USA
    Arch Neurol 65:636-41. 2008
    ....
  93. ncbi Regional distribution of TDP-43 inclusions in Alzheimer disease (AD) brains: their relation to AD common pathology
    Ai Kadokura
    Department of Neurology, Gunma University Graduate School of Medicine, Gunma, Japan
    Neuropathology 29:566-73. 2009
    ..We consider that it is still too early to determine that the TDP-43 accumulation is a part of AD pathology or result from a completely independent pathology...
  94. ncbi Formation of Hirano bodies in Dictyostelium and mammalian cells induced by expression of a modified form of an actin-crosslinking protein
    Andrew G Maselli
    Department of Cellular Biology, University of Georgia, Athens, GA 30602, USA
    J Cell Sci 115:1939-49. 2002
    ..The results reveal that formation of Hirano bodies is not necessarily related to cell death. These cultured cell models should facilitate studies of the biochemistry, genetics and physiological effects of Hirano bodies...
  95. ncbi Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia
    Fumiaki Mori
    Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, 5 Zaifu cho, Hirosaki, 036 8562, Japan
    Acta Neuropathol 116:193-203. 2008
    ..These findings suggest that there are differences in the formation process between skein-like and round inclusions, despite the antigenic and ultrastructural similarities...
  96. ncbi Activity of maize transglutaminase overexpressed in Escherichia coli inclusion bodies: an alternative to protein refolding
    Patricia Carvajal
    Dept of Molecular Genetics, Centre de Recerca Agrigenomica CRAG CSIC IRTA UAB, Jordi Girona Salgado 18 24, 08034 Barcelona, Spain
    Biotechnol Prog 27:232-40. 2011
    ..and overexpressed in Escherichia coli cells, and the recombinant protein (TGZp) was present mainly in inclusion bodies (IB) fraction...
  97. ncbi Refolding solubilized inclusion body proteins
    Richard R Burgess
    McArdle Laboratory for Cancer Research, University of Wisconsin Madison, Madison, Wisconsin, USA
    Methods Enzymol 463:259-82. 2009
    ..This chapter describes the main techniques and strategies for achieving successful refolding...
  98. ncbi Homeostatic levels of p62 control cytoplasmic inclusion body formation in autophagy-deficient mice
    Masaaki Komatsu
    Laboratory of Frontier Science, Tokyo Metropolitan Institute of Medical Science, Bunkyo ku, Tokyo 113 8613, Japan
    Cell 131:1149-63. 2007
    ....
  99. ncbi Macrophage lipid body induction by Chagas disease in vivo: putative intracellular domains for eicosanoid formation during infection
    R C N Melo
    Laboratory of Cellular Biology, Department of Biology, Federal University of Juiz de Fora, 36036 330, Juiz de Fora, MG, Brazil
    Tissue Cell 35:59-67. 2003
    ..The increase in LB numbers during infection positively correlates with increased generation of PGE(2), suggesting that LB may have a role in the heightened eicosanoid production observed during T. cruzi infection...
  100. ncbi Dopaminergic loss and inclusion body formation in alpha-synuclein mice: implications for neurodegenerative disorders
    E Masliah
    Department of Neurosciences, Department of Pathology, University of California San Diego, La Jolla, CA 92093 0624, USA
    Science 287:1265-9. 2000
    ..These results suggest that accumulation of wild-type alpha-synuclein may play a causal role in Parkinson's disease and related conditions...
  101. pmc BAG3 mediates chaperone-based aggresome-targeting and selective autophagy of misfolded proteins
    Martin Gamerdinger
    Institute of Pathobiochemistry, University Medical Center, Johannes Gutenberg University, Duesbergweg 6, Mainz 55099, Germany
    EMBO Rep 12:149-56. 2011
    ..Notably, aggresome-targeting by BAG3 is distinct from previously described mechanisms, as it does not depend on substrate ubiquitination...

Research Grants62

  1. Molecular Pathological Mechanism & Potential Therapeutics of Lafora Disease
    YAN NONE LIU; Fiscal Year: 2010
    ..myoclonus epilepsy (PME), severe neurological deterioration, and the accumulation of starch-like glycogen inclusion bodies (LB) mainly in the brain, muscle, and liver...
  2. Mitochondrial chaperones mortalin and Tid1 in protein degradation
    Carolyn K Suzuki; Fiscal Year: 2012
    ..Electron microscopy data show the accumulation of inclusion bodies within mitochondria of Lon-depleted cells...
  3. Cullin mediated protein turnover in skeletal and cardiac muscles
    Stephan Lange; Fiscal Year: 2012
    ..Accumulation of undegraded proteins in aggrosomes/inclusion bodies, or the premature degradation of (mutant) proteins is often associated with development of diseases, like ..
  4. Screening for inhibitors of M. tuberculosis persistence-related lipid metabolism
    Robert B Abramovitch; Fiscal Year: 2013
    ..In vitro growth conditions that induce DosR and NRP cause Mtb to accumulate inclusion bodies that are primarily composed of TAG...
  5. Cullin mediated protein turnover in skeletal and cardiac muscles
    Stephan Lange; Fiscal Year: 2013
    ..Accumulation of undegraded proteins in aggrosomes/inclusion bodies, or the premature degradation of (mutant) proteins is often associated with development of diseases, like ..
  6. Recombinant alpha1-Antitrypsin Therapeutics
    XINLI HENRY LIN; Fiscal Year: 2010
    ..coli inclusion bodies. PUBLIC HEALTH RELEVANCE: This project is to develop new mutant and peggylated forms of recombinant a1-..
  7. Deep Sequencing of Kawasaki Disease Tissues
    Anne H Rowley; Fiscal Year: 2012
    ..arterial immune response in acute KD, and synthetic versions of these antibodies identified intracytoplasmic inclusion bodies (ICI) in KD ciliated bronchial epithelium, in a subset of macrophages in lung, spleen, and lymph nodes, and ..
  8. The Role of TDP-43 Phosphorylation in Protein Function and Neurodegeneration
    YOUHNA AYALA; Fiscal Year: 2013
    ..TDP-43 is the main disease protein accumulating in cytoplasmic inclusion bodies in amyotrophic lateral sclerosis (ALS) and front temporal lobar degeneration (FTLD), two major forms of ..
  9. In vivo and crude extract analysis of polyQ aggregation intermediates
    Robert Fairman; Fiscal Year: 2012
    ..RELEVANCE: Twelve human diseases involve polyQ-driven aggregation, and the pathway to sequestration into inclusion bodies or nuclear inclusions has been shown to be complex, involving the accumulation of toxic oligomers...
  10. A new technology for preclinical development of functional bispecific antibodies
    Lee A Henderson; Fiscal Year: 2010
    ..BsAbs have emerged including hybrid hybridomas, chemical crosslinking, renaturation from bacterial inclusion bodies, and the use of non-covalent coupling in diabodies or single-chain antibody fragments (scFvs) with ..
  11. Pesticides and Parkinson's Disease
    Zhengui Xia; Fiscal Year: 2012
    ..in the substantia nigra pars compacta (SNpc) of the brain and formation of 1-synuclein containing inclusion bodies. Therefore, studies of paraquat-induced dopaminergic neuron death may provide important new information ..
  12. OXYGEN RADICAL TOXICITY AND PROTEIN DEGRADATION
    Kelvin J A Davies; Fiscal Year: 2013
    ..amp;structure, and must be proteolyticaly degraded or they will aggregate and form cross- linked cellular inclusion bodies. We have shown a major role for the Proteasome in detoxifying oxidized proteins...
  13. p53 Fusion Protein as an Oncology Therapeutic
    XINLI HENRY contact LIN; Fiscal Year: 2010
    ..coli inclusion bodies. In addition, we have demonstrated a proof of the concept that GnRH-p53 binds to GnRH receptors on the cell ..
  14. OXYGEN RADICAL TOXICITY AND PROTEIN DEGRADATION
    Kelvin J A Davies; Fiscal Year: 2012
    ..amp;structure, and must be proteolyticaly degraded or they will aggregate and form cross-linked cellular inclusion bodies. In previous grant cycles, we have shown a major role for the Proteasome in detoxifying oxidized proteins...
  15. Genetic Study of the Yeast Prion-Interacting Proteins
    Yury O Chernoff; Fiscal Year: 2010
    ..Accumulation of the large cytologically detectable aggregated structures (inclusion bodies) of prion proteins or other amyloidogenic proteins is observed in yeast in certain conditions and is ..
  16. Impaired Protein Degradation Pathways in Cardiac Proteinopathy
    Patrick M McLendon; Fiscal Year: 2013
    ..Our lab has identified the intracellular inclusion bodies formed by CryABR120G mutations as aggresomes, which have similar morphological features to the protein ..
  17. Molecular pathogenesis of SIMPLE in Charcot-Marie-Tooth disease
    MING HIN LEE; Fiscal Year: 2010
    ..myelin proteins, namely, peripheral myelin protein 22 (PMP22) and myelin protein zero (MPZ), to cytoplasmic inclusion bodies. These findings suggest that aberrant trafficking of PMP22 and MPZ are involved in CMT pathogenesis...
  18. Translational Studies in VCP Inclusion Body Myopathy with Paget Disease of Bone
    Virginia Kimonis; Fiscal Year: 2009
    ..model that demonstrates decreased muscle strength, disrupted muscle fibers, TDP-43 and ubiquitin positive inclusion bodies and vacuoles in muscle, cognitive deficits and Paget-like bone lesions...
  19. Biochemical Characterization of the Regulatory T-cell Protein LAG-3.
    Zarixia Zavala Ruiz; Fiscal Year: 2010
    ..We will continue our efforts in producing soluble functional LAG-3 by purifying it from inclusion bodies and refolding it in vitro as well as by expressing it in Drosphila Schneider-2 cells...
  20. Ferritin Induced Neurodegeneration
    Ruben Vidal; Fiscal Year: 2013
    ..HF is a movement disorder that is neuropathologically characterized by abnormal accumulation of ferritin inclusion bodies (IBs) and iron throughout the CNS (Vidal 2011)...
  21. Cellular models of microvillus inclusion disease
    H Lee Sweeney; Fiscal Year: 2013
    ..MVID or MID~ MIM#251850) is characterized by the loss of microvilli and the appearance of microvillus inclusion bodies in intestinal epithelial cells...
  22. ANESTHETIC MECHANISMS AND GABA-A RECEPTOR STRUCTURE
    MYLES AKABAS; Fiscal Year: 2001
    ..One hundred milligram quantities can be produced and purified from E. coli inclusion bodies. Conditions to refold the guanidine-solubilized protein will be investigated...
  23. Single-molecule study of alpha-synuclein structure modulation by interactions
    CRYSTAL ROSE MORAN-GUTIERREZ; Fiscal Year: 2013
    ..While the precise role played by a -syn in PD is unknown, its aggregation into filamentous inclusion bodies within the brain is the defining hallmark of this and other synucleinopathies...
  24. AUTOPHAGY IN GLIA AND NEURONS
    William Dunn; Fiscal Year: 1993
    ..neuronal ceroid lipofuscinosis (NCL), cerebral and retinal atrophy has been reported coincident with abnormal inclusion bodies found in astrocytes and neurons of the brain and pigmented epithelium of the retina...
  25. POLYGLUTAMINE CONFORMATION AND NEURODEGENERATION
    Steven M Finkbeiner; Fiscal Year: 2013
    ..huntingtin (htt) protein and characterized by aggregation of htt into visible intracellular structures called inclusion bodies (IBs)...
  26. Defining protein quality control for neurodegenerative disease-related proteins
    Emily Mitchell Sontag; Fiscal Year: 2013
    ..these triage decisions will further our knowledge of the mechanism of inclusion formation and the function of inclusion bodies within the cell...
  27. MECHANISMS OF HUNTINGTIN-INDUCED NEURODEGENERATION
    Steven M Finkbeiner; Fiscal Year: 2013
    ..to study a neuron model of HD and discovered that the aggregation of mHtt into visible deposits called inclusion bodies (IBs) appears to be a coping response...
  28. High Throughput Protein Production by Novel E. coli Expression-Secretion System
    WILLIAM MACCONNELL; Fiscal Year: 2006
    ..coli. These advantages include: a) it avoids the formation of inclusion bodies, which require subsequent dissolution and renaturation, b) secretion circumvents toxicity problems, as the ..
  29. C. Perfringens to Induce Anti-HIV Mucosal Immunity
    Phalguni Gupta; Fiscal Year: 2006
    ..perfringens construct expressing large amount of HIV-1 -1 gag p24 proteins in the form of inclusion bodies. Furthermore, dendritic cells (DCs) beneath the mucosal surface in the Peyer's Patches (PPs) captured ..
  30. Protein aggregation and inclusion body formation
    Ron R Kopito; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Accumulation of protein aggregates and ubiquitin (Ub) into cytoplasmic inclusion bodies (IB) is the single most definitive diagnostic neuropathological marker of neurodegenerative disease...
  31. Action tremor & dementia in male carriers of fragile X
    James Grigsby; Fiscal Year: 2007
    ..neurologic disorder consisting of intention tremor, ataxia, and dementia, with generalized brain atrophy and inclusion bodies, among older men with the fragile X premutation...
  32. TRANSPORT OF AN E COLI VIRULENCE FACTOR--THE K1 CAPSULE
    Richard Silver; Fiscal Year: 2000
    ..1) Does purified KpsT bind and hydrolyze ATP? Inclusion bodies from cells overexpressing KpsT will be solubilized with urea and the protein purified by dye-ligand ..
  33. INTERACTIONS BETWEEN ALPHA-SYNUCLEIN/METALS/PESTICIDES
    Donato Di Monte; Fiscal Year: 2003
    ..Perhaps most importantly, however, they will further our understanding of the relationship between inclusion bodies, dopaminergic degeneration and metals and pesticides, both of which have been implicated in the etiology of ..
  34. Recombinant Des-Kringle Derivatives of Plasmin for Treatment of Peripheral Arteri
    XLNLI LIN; Fiscal Year: 2006
    ..plasmin in their zymogenic form, mini-plasminogen (mini-Plg) and microplasminogen (mu Plg) recombinantly as inclusion bodies in an E...
  35. ADENOSINE RECEPTORS AND ION TRANSPORT IN EPITHELIA
    John Forrest; Fiscal Year: 2003
    ..loops confers the remarkable characteristic of Ao to be produced at high densities at high densities in inclusion bodies in E. coli...
  36. RAB GTP BINDING PROTEINS AND CELL GROWTH
    WILLIAM MALTESE; Fiscal Year: 2005
    ..was expressed in cultured cells, it appeared to induce a striking array of autophagic vacuoles and nuclear inclusion bodies. Based on these preliminary observations, the following hypotheses have been developed: (a) Rab24 normally ..
  37. FATAL HERPESVIRUS IN ELEPHANTS
    Laura Richman; Fiscal Year: 2002
    ..Ultrastructurally, the inclusion bodies seen by light microscopy are composed of viral particles measuring 80-92 nm in diameter, morphologically ..
  38. Viral inclusion bodies and aggresomes: form and function
    John Parker; Fiscal Year: 2004
    ..The research plan will test the hypothesis that the virus inclusion bodies (vlBs) that form in reovirus-infected cells co-opt the cellular misfolded protein response in order to ..
  39. Angiotensin and Neurodegeneration
    PATRICIA SONSALLA; Fiscal Year: 2009
    ..exhibit loss of striatal DA and tyrosine hydroxylase (TH), nigral DA cell death, microgliosis and striatal inclusion bodies. We will use this model to investigate if pharmacological manipulation of the brain renin angiotensin system ..
  40. PROTEINS IN LEAD-INDUCED NUCLEAR INCLUSION BODIES
    KEITH SHELTON; Fiscal Year: 1993
    ..The protein, p32/6.3, is an abundant component of lead- induced intranuclear inclusion bodies in kidney...
  41. Rational Optimization of Protein Solubility
    JOHN DESJARLAIS; Fiscal Year: 2003
    ..Furthermore, interferon beta forms inclusion bodies during expression in E. coli...
  42. Bronchial Epithelial Cultures and Kawasaki Disease
    Susan Baker; Fiscal Year: 2009
    ..Using light and transmission electron microscopy (TEM), we found that this antigen resides in cytoplasmic inclusion bodies consistent with aggregates of protein and associated nucleic acid...
  43. BACTERIAL EXPRESSION OF LYMPHOMA RNRNASE IMMUNOTOXIN
    SHUI ON LEUNG; Fiscal Year: 1999
    ..The proposal suggests the production of rpRNase or rpRNase-hLL2scFv in E. coli as inclusion bodies containing an N-terminal Methionine...
  44. Mitochrondrial Etiologies of Parkinsons Disease
    James Bennett; Fiscal Year: 2009
    ..Most dramatically, PD cybrids spontaneously generate Lewy inclusion bodies that are indistinguishable biochemically and morphologically from those present in PD brain...
  45. FUNCTION OF GFAP IN ASTROCYTE DEVELOPMENT AND DISEASE
    Albee Messing; Fiscal Year: 2002
    ..In contrast, the GFAP over-expressing mice develop astrocytes that are enlarged and contain complex inclusion bodies identical to the Rosenthal fibers seen in Alexander's disease...
  46. TRANSGLUTAMINASE AND DISEASES OF EXPANDED POLYGLUTAMINE
    Howard Green; Fiscal Year: 2002
    ..Further evidence will be sought to support all of these points by studies of 1 ) the purification of inclusion bodies containing expanded polyglutamine from other cellular components and their analysis for the presence of ( ..
  47. Niemann Pick C disease in Drosophila
    Krishna Bhat; Fiscal Year: 2007
    ..flies show progressive, age-dependent locomotor deficits, (2) the brains from these mutants show neurons with inclusion bodies similar to the inclusions found in neurons of NPC and several other lysosomal storage disorders...
  48. Hook proteins in membrane trafficking & neurogeneration
    HELMUT KRAMER; Fiscal Year: 2006
    ..Instead, they are concentrated in inclusion bodies. How these inclusion bodies are linked to the progression of neurodegenerative diseases is not well ..
  49. Formation and toxicity of peripherin inclusions
    Jean Pierre Julien; Fiscal Year: 2004
    ..The majority of these inclusion bodies are composed of neurofilament (NF) proteins together with peripherin, a type III IF normally expressed at low ..
  50. Bioengineering Prourokinase for Improved Fibrinolysis
    DANIEL MEDYNSKI; Fiscal Year: 2004
    ..The bioengineered variants of proUK will be expressed in E. coli and refolded and purified from insoluble inclusion bodies. We have already refolded and purified wild type proUK from inclusion bodies, and demonstrated that upon ..
  51. ASSOC/FOLDING PROCESSES OF THIOREDOXIN FRAGMENT
    MARIA TASAYCO; Fiscal Year: 2000
    ..primary sequence, the design of denovo proteins with desired properties, and the refolding of proteins from inclusion bodies, all require a clear understanding of the principles underlying the formation and assembly of alpha-helices ..
  52. P62, Protein Aggregation and Fatty Liver Disease
    Martin Obin; Fiscal Year: 2005
    ..These degenerative diseases exhibit characteristic "inclusion bodies," which are enriched for aggregates of ubiquitinated, misfolded protein and p62/sequestosome-1, a ubiquitin ..
  53. FOLDING MECHANISM OF A BETA PROTEIN
    Patricia Jennings; Fiscal Year: 2000
    ..the lifetimes and/or solubilities of transient intermediates in the folding pathway, the tendency to form inclusion bodies will be studied by kinetic analysis...
  54. BIOCHEMICAL AND MOLECULAR BASIS OF THE COMP DISORDERS
    Daniel Cohn; Fiscal Year: 2003
    ..Diagnostic radiographic abnormalities of the epiphyses and metaphyses, as well as unique inclusion bodies within chondrocytes define the condition...
  55. Tissue Transglutaminase in Neurologic Injury
    THUNG SHEN LAI; Fiscal Year: 2007
    ..soluble high molecular weight (HMW) oligomeric complexes are the neurotoxic species whereas the insoluble inclusion bodies are a protective reservoir of non-toxic aggregates...
  56. Mechanism of Notch Transcriptional Activation
    Katherine Jones; Fiscal Year: 2006
    ..This region of MAM also mobilizes CBP/p300 to nuclear inclusion bodies similar to those formed by mutant polyglutamine-repeat proteins in neurodegenerative diseases...
  57. Generation of Membrane Protein Production Strains
    JAMES BOWIE; Fiscal Year: 2009
    ..can improve the production of poorly expressed membrane proteins and/or that redirect expression from inclusion bodies into membranes. Aim II. Identify the genes/mutations. Aim III...
  58. FENIB: A familial seizure disorder
    PETER HOLOHAN; Fiscal Year: 2003
    ..disease causing both epilepsy and progressive dementia called familial encephalopathy with neuroserpin inclusion bodies (FENIB)...
  59. A Chaperonin/Osmolyte Protein Folding Screen - STTR Phase I
    Mark Fisher; Fiscal Year: 2007
    ..This system can successfully fold 1) aggregation prone proteins, 2) insoluble proteins isolated from inclusion bodies, 3) misfolded soluble proteins, and 4) misfolded proteins found in disease states...
  60. AGGREGATION OF PROTEINS IN AGE-RELATED DISEASES
    Michael Sherman; Fiscal Year: 2005
    ..These toxic abnormal proteins can aggregate in cells and form large inclusion bodies, and there is an ongoing discussion in the field how aggregation of these proteins influences neurotoxicity...
  61. The Role of Parkin in the Synaptic Vesicle Cycle
    Kristen Willeumier; Fiscal Year: 2007
    ..in PARK2; however, pathological evaluation of neural tissue reveals the absence of intracytoplasmic inclusion bodies that typically result from excess aggregate buildup...
  62. NEUROSERPIN STORAGE DISORDER WITH DEMENTIA
    PETER HOLOHAN; Fiscal Year: 1999
    ..The investigation of the disorder was advanced after the neuronal inclusion bodies that are its distinguishing pathological feature were isolated and were found to be composed primarily of ..