Mutant PolyQ Can Aggregate in the Heart , Causing Cardiac Dysfunction and Myo fi brillar Degradation

Drosophila as a potential model to ameliorate mutant Huntington-mediated cardiac amyloidosis Adriana S Trujillo, Raul Ramos, Rolf Bodmer, Sanford I Bernstein, Karen Ocorr & Girish C Melkani a Department of Biology; Molecular Biology and Heart Institutes; San Diego State University; San Diego, CA USA b Development and Aging Program; Sanford-Burnham Institute for Medical Research; La Jolla, CA USA Published online: 03 Nov 2014.

[1]  Karen Ocorr,et al.  Huntington's Disease Induced Cardiac Amyloidosis Is Reversed by Modulating Protein Folding and Oxidative Stress Pathways in the Drosophila Heart , 2013, PLoS genetics.

[2]  R. Petersen,et al.  Inhibiting toxic aggregation of amyloidogenic proteins: a therapeutic strategy for protein misfolding diseases. , 2013, Biochimica et biophysica acta.

[3]  B. Edgar,et al.  Suppression of polyglutamine protein toxicity by co-expression of a heat-shock protein 40 and a heat-shock protein 110 , 2013, Cell Death and Disease.

[4]  M. Maeda,et al.  Prefoldin Protects Neuronal Cells from Polyglutamine Toxicity by Preventing Aggregation Formation* , 2013, The Journal of Biological Chemistry.

[5]  R. Talmadge,et al.  Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction , 2013, Proceedings of the National Academy of Sciences.

[6]  Daphne Merkus,et al.  Reactive Oxygen Species and the Cardiovascular System , 2013, Oxidative medicine and cellular longevity.

[7]  T. Clausen,et al.  The Myosin Chaperone UNC-45 Is Organized in Tandem Modules to Support Myofilament Formation in C. elegans , 2013, Cell.

[8]  S. Finkbeiner,et al.  Protein aggregates in Huntington's disease , 2012, Experimental Neurology.

[9]  A. Hannan,et al.  Neurocardiac dysregulation and neurogenic arrhythmias in a transgenic mouse model of Huntington's disease , 2012, The Journal of physiology.

[10]  R. Roos,et al.  Aspiration pneumonia and death in Huntington’s disease , 2012, PLoS currents.

[11]  K. Ocorr,et al.  The UNC-45 Chaperone Is Critical for Establishing Myosin-Based Myofibrillar Organization and Cardiac Contractility in the Drosophila Heart Model , 2011, PloS one.

[12]  P. Hemachandra Reddy,et al.  Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage. , 2011, Human molecular genetics.

[13]  S. Bernstein,et al.  Drosophila UNC-45 accumulates in embryonic blastoderm and in muscles, and is essential for muscle myosin stability , 2011, Journal of Cell Science.

[14]  Ernesto Carafoli,et al.  Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli , 2010, EMBO molecular medicine.

[15]  Jody Groenendyk,et al.  Biology of endoplasmic reticulum stress in the heart. , 2010, Circulation research.

[16]  C. Rodolfo,et al.  Proteomic analysis of mitochondrial dysfunction in neurodegenerative diseases , 2010, Expert review of proteomics.

[17]  Shihua Li,et al.  Polyglutamine toxicity in non-neuronal cells , 2010, Cell Research.

[18]  Richard T. Lee,et al.  Biochemical and Mechanical Dysfunction in a Mouse Model of Desmin-Related Myopathy , 2009, Circulation research.

[19]  J. Robbins,et al.  Protein misfolding and cardiac disease: Establishing cause and effect , 2008, Autophagy.

[20]  J. Robbins,et al.  Cardiomyocyte Expression of a Polyglutamine Preamyloid Oligomer Causes Heart Failure , 2008, Circulation.

[21]  D. Winkelmann,et al.  Unc45b Forms a Cytosolic Complex with Hsp90 and Targets the Unfolded Myosin Motor Domain , 2008, PloS one.

[22]  D. Pilgrim,et al.  The myosin co-chaperone UNC-45 is required for skeletal and cardiac muscle function in zebrafish. , 2007, Developmental biology.

[23]  K. Hoyt,et al.  Cardiac dysfunction in the R6/2 mouse model of Huntington’s disease , 2007, Neurobiology of Disease.

[24]  Karen Ocorr,et al.  Age-related cardiac disease model of Drosophila , 2007, Mechanisms of Ageing and Development.

[25]  D. Maass,et al.  Cardiac mitochondrial damage and loss of ROS defense after burn injury: the beneficial effects of antioxidant therapy. , 2007, Journal of applied physiology.

[26]  N. Nukina,et al.  Oxidative stress promotes mutant huntingtin aggregation and mutant huntingtin-dependent cell death by mimicking proteasomal malfunction. , 2006, Biochemical and biophysical research communications.

[27]  E. Olson,et al.  Hand, an evolutionarily conserved bHLH transcription factor required for Drosophila cardiogenesis and hematopoiesis , 2006, Development.

[28]  Joseph A Izatt,et al.  Drosophila as a model for the identification of genes causing adult human heart disease , 2006, Proceedings of the National Academy of Sciences of the United States of America.

[29]  ElizabethMurphy,et al.  Mitochondrial Dysfunction and Apoptosis Underlie the Pathogenic Process in α-B-Crystallin Desmin-Related Cardiomyopathy , 2005 .

[30]  Sarah J Tabrizi,et al.  Gene expression in Huntington's disease skeletal muscle: a potential biomarker. , 2005, Human molecular genetics.

[31]  R. Bodmer,et al.  Drosophila, an emerging model for cardiac disease. , 2004, Gene.

[32]  J. Saffitz,et al.  Desmin-related cardiomyopathy in transgenic mice: a cardiac amyloidosis. , 2004, Proceedings of the National Academy of Sciences of the United States of America.

[33]  J. Turrens,et al.  Mitochondrial formation of reactive oxygen species , 2003, The Journal of physiology.

[34]  F. Hartl,et al.  Role of the Myosin Assembly Protein UNC-45 as a Molecular Chaperone for Myosin , 2002, Science.

[35]  T. Hewett,et al.  Expression of R120G-alphaB-crystallin causes aberrant desmin and alphaB-crystallin aggregation and cardiomyopathy in mice. , 2001, Circulation research.

[36]  G. Sobue,et al.  Chaperones Hsp70 and Hsp40 Suppress Aggregate Formation and Apoptosis in Cultured Neuronal Cells Expressing Truncated Androgen Receptor Protein with Expanded Polyglutamine Tract* , 2000, The Journal of Biological Chemistry.

[37]  M. Beal,et al.  Mitochondrial dysfunction and oxidative stress in aging and neurodegenerative disease. , 2000, Journal of neural transmission. Supplementum.

[38]  Douglas C. Wallace,et al.  A novel neurological phenotype in mice lacking mitochondrial manganese superoxide dismutase , 1998, Nature Genetics.

[39]  B. Rah,et al.  Quantitative study on the relation between structural and functional properties of the hearts from three different mammals , 1994, The Anatomical record.

[40]  S. Folstein,et al.  Huntington's disease gene (IT15) is widely expressed in human and rat tissues , 1993, Neuron.

[41]  K. Fenger,et al.  Causes of death in patients with Huntington's disease and in unaffected first degree relatives. , 1992, Journal of medical genetics.

[42]  J. Schaper,et al.  Ultrastructural quantitation of mitochondria and myofilaments in cardiac muscle from 10 different animal species including man. , 1992, Journal of molecular and cellular cardiology.

[43]  D. Lanska,et al.  Huntington's disease mortality in the United States , 1988, Neurology.

[44]  E. Chiu,et al.  Causes of death in Huntington's Disease , 1982, The Medical journal of Australia.