glutamine and Huntington Disease

glutamine has been researched along with Huntington Disease in 70 studies

Research

Studies (70)

TimeframeStudies, this research(%)All Research%
pre-19908 (11.43)18.7374
1990's21 (30.00)18.2507
2000's31 (44.29)29.6817
2010's9 (12.86)24.3611
2020's1 (1.43)2.80

Authors

AuthorsStudies
Al-Harrasi, A; Aleya, L; Alsubayiel, AM; Behl, T; Bhatia, S; Dailah, HG; Fuloria, NK; Fuloria, S; Naved, T; Sachdeva, M; Sekar, M; Sharma, N; Singh, S; Subramaniyan, V; Zahoor, I1
Aldana, BI; Andersen, JV; Nielsen, ML; Nørremølle, A; Santos, A; Skotte, NH; Waagepetersen, HS; Willert, CW1
Long, JD; Paulsen, JS; Smith, MM1
Bano, D; Floriddia, EM; Jakubik, M; Nicotera, P1
Castro, LM; Dasgupta, S; Ferro, ES; Fishman, MA; Fricker, LD; Mahallati, H; Tashima, AK1
Ashkan, K; Cho, NJ; Inayathullah, M; Jeyaraj, R; Lam, J; Liu, CW; Mahmoudi, M; Manoukian, MA; Rajadas, J; Tan, A1
Déglon, N; Jollé, C; Merienne, N; Meunier, C; Pellerin, L1
Furukawa, Y; Kaneko, K; Kurosawa, M; Matsumoto, G; Nukina, N1
Barrow, RK; Mealer, RG; Sixt, KM; Snyder, SH; Subramaniam, S; Usiello, A1
Orr, HT2
Gabery, S; Hult, S; Kirik, D; Petersén, Å; Sajjad, MU; Soylu, R1
Behrens, PF; Franz, P; Landwehrmeyer, GB; Lindenberg, KS; Woodman, B1
Cattaneo, E; Rigamonti, D; Zuccato, C1
Goellner, GM; Rechsteiner, M1
Flumerfelt, BA; Hussain, N; Rajakumar, N1
Aasly, J; Gårseth, M; Sonnewald, U; White, LR1
Mark, LP; Prost, RW; Reynolds, NC1
Auer, DP; Bender, A; Bender, J; Dose, M; Gasser, T; Klopstock, T; Merl, T; Reilmann, R; Saemann, P; Weindl, A; Yassouridis, A1
Abderrahmane, S; Arango, M; Catoire, H; Lambert, E; Néri, C; Parker, AJ; Tourette, C1
de Almeida, LP; Rego, AC1
Crouse, AB; Detloff, PJ1
Bates, GP1
Brusilow, WS1
Buescher, JL; Carrier, RL; Funk, JA; Hoyt, KR; Ma, TC; Nash, AJ; Oatis, B1
Atwal, RS; Burtnik, A; Truant, R1
Aebischer, P; Bloch, J; Brouillet, E; Condé, F; Déglon, N; Hantraye, P; Jan, C; Jarraya, B; Li, XJ; Palfi, S; Shin, M1
Hayashi, H; Kagamihara, Y; Kawata, A; Matsubara, S; Sugaya, K1
Duan, W; Jiang, M; Li, Q; Liang, Y; Masuda, N; Peng, Q; Ross, CA; Wang, W; Wang, X; Zhao, M1
Cadet, JL; Duan, W; Ford, E; Ladenheim, B; Masuda, N; Peng, Q; Ross, CA; Tryggestad, E; Wong, J; Zhao, M1
Blass, JP; Cooper, AJ; Folk, JE; Jeitner, TM; Matson, WR1
Carter, CJ2
Mangano, RM; Schwarcz, R1
Archer, FJ; Mancall, EL1
Beverstock, GC1
Elkins, J; Gray, PN; May, PC; Mundy, L1
Cancel, G; David, G; Devys, D; Imbert, G; Lutz, Y; Saudou, F; Stevanin, G; Tora, L; Trottier, Y; Weber, C1
Li, SH; Loev, SJ; Ross, CA; Schilling, G; Sharp, AH; Stine, OC; Wagster, MV1
Lange, HW; Reilmann, R; Rolf, LH1
Brooks, DJ; Bryant, DJ; Harding, AE; Marcus, CD; Sargentoni, J; Taylor-Robinson, SD; Weeks, RA2
Pearson, SJ; Reynolds, GP1
Maddox, J1
Finch, JT; Johnson, T; Perutz, MF; Suzuki, M1
Hornykiewicz, O1
Perutz, MF1
Crow, TJ; DeLisi, LE; Jain, S; Leggo, J; Paykel, ES; Rubinsztein, DC; Walsh, C1
Amaechi, I; Bates, G; Mangiarini, L; Sathasivam, K1
Ashworth, RG; Li, SH; Loev, SJ; Margolis, RL; Ross, CA; Schilling, G; Young, WS1
Johnson, T1
Li, SH; Li, XJ1
Borchelt, DR; Cooper, JK; Dawson, TM; Dawson, VL; Delanoy, M; Herring, WJ; Kaminsky, Z; Khan, FA; Masone, J; Peters, MF; Ross, CA; Schilling, G; Sharp, AH1
Mitchell, A1
Bates, GP; Eickhoff, H; Hasenbank, R; Lehrach, H; Scherzinger, E; Sittler, A; Wälter, S; Wanker, EE; Wedemeyer, N1
Cooper, J; Greenamyre, JT; Lawler, JF; Margolis, RL; Ross, CA; Sawa, A; Sharp, AH; Snyder, SH; Wiegand, GW1
Peters, MF; Ross, CA1
Auerbach, W; Gusella, JF; Gutekunst, CA; Hersch, S; Joyner, AL; Li, SH; Li, XJ; MacDonald, ME; Vonsattel, JP; Vrbanac, V; Weaver, M; Wheeler, VC; White, JK; Yi, H1
Andreassen, OA; Beal, MF; Ferrante, RJ; Jenkins, BG; Klivenyi, P; Kustermann, E; Rosen, BR1
Bates, G1
Orr, HT; Zoghbi, HY1
Dausset, J; Denghien, I; Ferrante, RJ; Hayden, MR; Holbert, S; Kiechle, T; Margolis, RL; Néri, C; Rosenblatt, A; Ross, CA; Wellington, C1
Perutz, MF; Windle, AH1
Apostol, BL; Bodai, L; Greenwald, M; Housman, DE; Jackson, GR; Kazantsev, A; Kurokawa, R; Marsh, JL; McCampbell, A; Pallos, J; Poelman, M; Schmidt, E; Steffan, JS; Thompson, LM; Zhu, YZ1
Liu, YF; Perides, G; Song, C1
Borchelt, DR; Friedlander, RM; Gusella, JF; Gutekunst, CA; Hersch, S; Lebel, LA; MacDonald, ME; Schilling, G; Vonsattel, JP; Vrbanac, V; Wheeler, VC1
Berthelier, V; Chen, S; Hamilton, JB; O'Nuallain, B; Wetzel, R1
Hung, W; Tourian, A1
Beal, MF; Finn, SF; Kowall, NW; Mazurek, MF; Storey, E1
Gerstenbrand, F; Gründig, E; Schedl, R; Simanyi, M; Weiss, H1

Reviews

10 review(s) available for glutamine and Huntington Disease

ArticleYear
Focusing the pivotal role of nanotechnology in Huntington's disease: an insight into the recent advancements.
    Environmental science and pollution research international, 2022, Volume: 29, Issue:49

    Topics: Glutamine; Humans; Huntington Disease; Liposomes; Nanoparticles; Nanotechnology

2022
Polyglutamine neurodegeneration: expanded glutamines enhance native functions.
    Current opinion in genetics & development, 2012, Volume: 22, Issue:3

    Topics: Animals; Autoantigens; Cell Cycle Proteins; Cell Nucleus; Cilia; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Nerve Tissue Proteins; Peptides; Phosphorylation; Receptors, Androgen; Spinocerebellar Ataxias; Structure-Activity Relationship; Trinucleotide Repeat Expansion

2012
Are Huntington's and polyglutamine-based ataxias proteasome storage diseases?
    The international journal of biochemistry & cell biology, 2003, Volume: 35, Issue:5

    Topics: Animals; Ataxia; Autoantigens; Cysteine Endopeptidases; Glutamine; Huntington Disease; Mice; Multienzyme Complexes; Nuclear Proteins; Proteasome Endopeptidase Complex; Proteins; Ubiquitin

2003
Molecular targets and therapeutic strategies in Huntington's disease.
    Current drug targets. CNS and neurological disorders, 2005, Volume: 4, Issue:4

    Topics: Animals; Axonal Transport; Caspases; DNA Repeat Expansion; Energy Metabolism; Gene Silencing; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Inclusion Bodies; Mitochondria; Neostriatum; Nerve Degeneration; Nerve Tissue Proteins; Neurons; Neurotoxins; Nuclear Proteins; Peptides; Protein Transport; Rats; Receptors, N-Methyl-D-Aspartate

2005
Is Huntington's a glutamine storage disease?
    The Neuroscientist : a review journal bringing neurobiology, neurology and psychiatry, 2006, Volume: 12, Issue:4

    Topics: Animals; Biological Evolution; Brain; Brain Diseases, Metabolic, Inborn; Glutamic Acid; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Nerve Tissue Proteins; Nuclear Proteins; Peptides

2006
Nucleocytoplasmic trafficking and transcription effects of huntingtin in Huntington's disease.
    Progress in neurobiology, 2007, Volume: 83, Issue:4

    Topics: Active Transport, Cell Nucleus; Cell Nucleus; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Nerve Tissue Proteins; Nuclear Proteins; Peptides; Transcription, Genetic

2007
The current state of research with peripheral tissues in Huntington disease.
    Human genetics, 1984, Volume: 66, Issue:2-3

    Topics: Antibody-Dependent Cell Cytotoxicity; Biopsy; Blood Platelets; Cell Division; Cell Membrane; Cell Migration Inhibition; Cells, Cultured; Chromosome Aberrations; Electron Spin Resonance Spectroscopy; Erythrocyte Membrane; Female; Fibroblasts; gamma-Aminobutyric Acid; Glutamine; Humans; Huntington Disease; Immunoglobulin G; Karyotyping; Leukocytes; Male; Mutation; Radiation Tolerance; Skin; Spectrometry, Fluorescence

1984
Parkinson's disease and the adaptive capacity of the nigrostriatal dopamine system: possible neurochemical mechanisms.
    Advances in neurology, 1993, Volume: 60

    Topics: Corpus Striatum; Dopamine; Glutamine; Humans; Huntington Disease; Lesch-Nyhan Syndrome; Malignant Hyperthermia; Neurons; Parkinson Disease; Receptors, Dopamine; Receptors, Glutamate; Substantia Nigra

1993
Glutamine repeats and neurodegeneration.
    Annual review of neuroscience, 2000, Volume: 23

    Topics: Animals; Glutamine; Humans; Huntington Disease; Muscular Disorders, Atrophic; Neurodegenerative Diseases; Peptides; Repetitive Sequences, Amino Acid; Spinocerebellar Ataxias

2000
Beyond the Qs in the polyglutamine diseases.
    Genes & development, 2001, Apr-15, Volume: 15, Issue:8

    Topics: Cysteine Endopeptidases; Glutamine; Humans; Huntington Disease; Multienzyme Complexes; Mutation; Peptides; Proteasome Endopeptidase Complex; Spinocerebellar Ataxias; Ubiquitins

2001

Trials

1 trial(s) available for glutamine and Huntington Disease

ArticleYear
Creatine supplementation lowers brain glutamate levels in Huntington's disease.
    Journal of neurology, 2005, Volume: 252, Issue:1

    Topics: Administration, Oral; Adult; Aspartic Acid; Brain; Cerebral Cortex; Creatine; Down-Regulation; Energy Metabolism; Female; Glutamic Acid; Glutamine; Humans; Huntington Disease; Magnetic Resonance Spectroscopy; Male; Middle Aged; Neurologic Examination; Neuroprotective Agents; Pilot Projects; Treatment Outcome

2005

Other Studies

59 other study(ies) available for glutamine and Huntington Disease

ArticleYear
Integrative Characterization of the R6/2 Mouse Model of Huntington's Disease Reveals Dysfunctional Astrocyte Metabolism.
    Cell reports, 2018, 05-15, Volume: 23, Issue:7

    Topics: 3-Hydroxybutyric Acid; Acetates; Animals; Astrocytes; Brain; Disease Models, Animal; Energy Metabolism; Female; Glutamic Acid; Glutamine; Humans; Huntington Disease; Male; Mice, Transgenic; Neurotransmitter Agents; Proteome

2018
Cognitive decline in prodromal Huntington Disease: implications for clinical trials.
    Journal of neurology, neurosurgery, and psychiatry, 2013, Volume: 84, Issue:11

    Topics: Adult; Clinical Trials as Topic; Cognition Disorders; Disease Progression; DNA Mutational Analysis; Feasibility Studies; Female; Genetic Testing; Glutamine; Humans; Huntington Disease; Longitudinal Studies; Male; Middle Aged; Neurologic Examination; Neuropsychological Tests; Outcome Assessment, Health Care; Prodromal Symptoms; Trinucleotide Repeat Expansion

2013
Heterozygous Igf1r deletion does not ameliorate pathological features associated with polyglutamine-containing huntingtin fragment.
    Neuroscience letters, 2014, Sep-19, Volume: 580

    Topics: Animals; Glutamine; Heterozygote; Humans; Huntingtin Protein; Huntington Disease; Mice, Knockout; Nerve Tissue Proteins; Peptides; Protein Aggregates; Receptor, IGF Type 1

2014
Reduced Levels of Proteasome Products in a Mouse Striatal Cell Model of Huntington's Disease.
    PloS one, 2015, Volume: 10, Issue:12

    Topics: Animals; Bortezomib; Cell Line; Corpus Striatum; Disease Models, Animal; Glutamine; Huntingtin Protein; Huntington Disease; Mice; Nerve Tissue Proteins; Nuclear Proteins; Oligopeptides; Peptides; Proteasome Endopeptidase Complex; Proteasome Inhibitors; Proteomics

2015
Self-assembly and sequence length dependence on nanofibrils of polyglutamine peptides.
    Neuropeptides, 2016, Volume: 57

    Topics: Animals; Fluorescence Resonance Energy Transfer; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Magnetic Resonance Spectroscopy; Microscopy, Atomic Force; Peptides; Protein Folding; Spectroscopy, Fourier Transform Infrared

2016
Astrocytes are key but indirect contributors to the development of the symptomatology and pathophysiology of Huntington's disease.
    Glia, 2016, Volume: 64, Issue:11

    Topics: Animals; Astrocytes; Brain; Cyclophilin A; Disease Models, Animal; DNA-Binding Proteins; Dopamine and cAMP-Regulated Phosphoprotein 32; Gene Expression Regulation; Glial Fibrillary Acidic Protein; Glutamate-Ammonia Ligase; Glutamic Acid; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Locomotion; Mice; Mice, Transgenic; Mutation; Nerve Tissue Proteins; Neurons; Nuclear Proteins

2016
Cross-seeding fibrillation of Q/N-rich proteins offers new pathomechanism of polyglutamine diseases.
    The Journal of neuroscience : the official journal of the Society for Neuroscience, 2009, Apr-22, Volume: 29, Issue:16

    Topics: Animals; Asparagine; Cell Line, Tumor; Cells, Cultured; Disease Models, Animal; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Intranuclear Inclusion Bodies; Mice; Mice, Transgenic; Nerve Tissue Proteins; Nuclear Proteins; Peptides; Protein Structure, Tertiary; Protein Transport; RNA-Binding Proteins; Solubility

2009
Rhes, a physiologic regulator of sumoylation, enhances cross-sumoylation between the basic sumoylation enzymes E1 and Ubc9.
    The Journal of biological chemistry, 2010, Jul-02, Volume: 285, Issue:27

    Topics: Animals; Corpus Striatum; Cysteine; Glutamine; GTP-Binding Proteins; Humans; Huntington Disease; Lysine; Mice; Mice, Knockout; Mutation; Small Ubiquitin-Related Modifier Proteins; Ubiquitin-Conjugating Enzymes; Ubiquitin-Protein Ligases

2010
Characterization of a rat model of Huntington's disease based on targeted expression of mutant huntingtin in the forebrain using adeno-associated viral vectors.
    The European journal of neuroscience, 2012, Volume: 36, Issue:6

    Topics: Animals; Body Weight; Brain-Derived Neurotrophic Factor; Dependovirus; Disease Models, Animal; Female; Genetic Vectors; Glutamine; Huntingtin Protein; Huntington Disease; Hypothalamus; Inclusion Bodies; Intracellular Signaling Peptides and Proteins; Male; Motor Activity; Mutation; Nerve Tissue Proteins; Neuropeptides; Nuclear Proteins; Orexins; Prosencephalon; Rats; Rats, Sprague-Dawley

2012
Impaired glutamate transport and glutamate-glutamine cycling: downstream effects of the Huntington mutation.
    Brain : a journal of neurology, 2002, Volume: 125, Issue:Pt 8

    Topics: Aging; Animals; Biological Transport; Brain; Cerebral Cortex; Chromatography, High Pressure Liquid; Corpus Striatum; Disease Models, Animal; Excitatory Amino Acid Transporter 2; Female; Glutamic Acid; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Immunohistochemistry; Male; Mice; Mice, Transgenic; Microdialysis; Mutation; Nerve Tissue Proteins; Nuclear Proteins

2002
The enigma of Huntington's disease.
    Scientific American, 2002, Volume: 287, Issue:6

    Topics: Animals; Brain; Brain Chemistry; Brain-Derived Neurotrophic Factor; Chromosomes, Human, Pair 4; Ciliary Neurotrophic Factor; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Middle Aged; Mutation; Nerve Tissue Proteins; Nuclear Proteins; Peptides; Repetitive Sequences, Nucleic Acid

2002
Glutamatergic regulation of long-term grafts of fetal lateral ganglionic eminence in a rat model of Huntington's disease.
    Neurobiology of disease, 2004, Volume: 15, Issue:3

    Topics: Animals; Brain Injuries; Brain Tissue Transplantation; Corpus Striatum; Disease Models, Animal; Dizocilpine Maleate; Dopamine Antagonists; Excitatory Amino Acid Antagonists; Fetal Tissue Transplantation; Fluorescent Antibody Technique; Glutamine; Haloperidol; Huntington Disease; Immunohistochemistry; Male; Neurons, Afferent; Proto-Oncogene Proteins c-fos; Quinolinic Acid; Rats; Rats, Sprague-Dawley; Receptors, Dopamine D2; Receptors, N-Methyl-D-Aspartate

2004
Cerebrospinal fluid from patients with dementia contains increased amounts of an unknown factor.
    Journal of neuroscience research, 2004, Oct-15, Volume: 78, Issue:2

    Topics: Adult; Aged; Aged, 80 and over; Alzheimer Disease; Amyotrophic Lateral Sclerosis; Case-Control Studies; Choline; Dementia, Vascular; Female; Glutamine; Humans; Huntington Disease; Magnetic Resonance Spectroscopy; Male; Middle Aged; Parkinson Disease

2004
Heterogeneity in 1H-MRS profiles of presymptomatic and early manifest Huntington's disease.
    Brain research, 2005, Jan-07, Volume: 1031, Issue:1

    Topics: Adult; Age of Onset; Aged; Aspartic Acid; Early Diagnosis; Female; Glutamic Acid; Glutamine; Humans; Huntington Disease; Magnetic Resonance Spectroscopy; Male; Middle Aged; Protons; Putamen

2005
[Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons].
    Medecine sciences : M/S, 2005, Volume: 21, Issue:5

    Topics: Animals; Disease Models, Animal; Glutamine; Huntington Disease; Mammals; Mutation; Nematoda; Peptides; Resveratrol; Sirtuins; Stilbenes

2005
Allele-specific conditional destabilization of glutamine repeat mRNAs.
    Gene expression, 2005, Volume: 12, Issue:3

    Topics: Alleles; Animals; Base Sequence; Codon; Dose-Response Relationship, Drug; Embryo, Mammalian; Gene Expression Regulation; Genome, Human; Glutamine; Humans; Huntington Disease; Mice; Models, Genetic; Models, Theoretical; Molecular Sequence Data; Peptides; Protein Biosynthesis; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger; RNA, Transfer; Stem Cells; Time Factors; Trinucleotide Repeat Expansion; Trinucleotide Repeats

2005
BIOMEDICINE: One Misfolded Protein Allows Others to Sneak By.
    Science (New York, N.Y.), 2006, Mar-10, Volume: 311, Issue:5766

    Topics: Animals; Caenorhabditis elegans; Caenorhabditis elegans Proteins; Disease Models, Animal; Glutamine; Humans; Huntington Disease; Mutation; Neurodegenerative Diseases; Peptides; Protein Conformation; Protein Folding; Temperature

2006
Metformin therapy in a transgenic mouse model of Huntington's disease.
    Neuroscience letters, 2007, Jan-10, Volume: 411, Issue:2

    Topics: Age Factors; AMP-Activated Protein Kinases; Animals; Behavior, Animal; Blood Glucose; Disease Models, Animal; Dose-Response Relationship, Drug; Enzyme Activation; Female; Glutamine; Huntington Disease; Hypoglycemic Agents; Male; Metformin; Mice; Mice, Transgenic; Motor Activity; Multienzyme Complexes; Protein Serine-Threonine Kinases; Sex Factors; Trinucleotide Repeats

2007
Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates.
    Molecular therapy : the journal of the American Society of Gene Therapy, 2007, Volume: 15, Issue:8

    Topics: Animals; Biomarkers; Dopamine; Gene Expression; Genetic Vectors; Glutamine; Huntington Disease; Macaca fascicularis; Male; Motor Neuron Disease; Movement; Mutation; Nerve Tissue Proteins; Nuclear Proteins; Peptide Fragments; Phenotype; Putamen

2007
Polyglutamine expansion mutation yields a pathological epitope linked to nucleation of protein aggregate: determinant of Huntington's disease onset.
    PloS one, 2007, Jul-25, Volume: 2, Issue:7

    Topics: Age of Onset; Alzheimer Disease; Cell Death; DNA; DNA Primers; DNA Repeat Expansion; Genes, Reporter; Glutamine; Humans; Huntington Disease; Kinetics; Mutation; Parkinson Disease; Peptides; Plasmids; Polylysine; Polymerase Chain Reaction; Transfection

2007
Tiagabine is neuroprotective in the N171-82Q and R6/2 mouse models of Huntington's disease.
    Neurobiology of disease, 2008, Volume: 30, Issue:3

    Topics: Animals; Arginine; Asparagine; Disease Models, Animal; Female; Glutamine; Huntington Disease; Male; Mice; Mice, Inbred C3H; Mice, Inbred C57BL; Mice, Inbred CBA; Mice, Neurologic Mutants; Mice, Transgenic; Neuroprotective Agents; Nipecotic Acids; PC12 Cells; Rats; Tiagabine

2008
Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's disease.
    Neurobiology of disease, 2008, Volume: 30, Issue:3

    Topics: Animals; Asparagine; Cell Differentiation; Cell Movement; Disease Models, Animal; Disease Progression; Glutamine; Huntington Disease; Male; Mice; Mice, Inbred C3H; Mice, Inbred C57BL; Mice, Transgenic; Neurons; Sertraline

2008
Increased levels of gamma-glutamylamines in Huntington disease CSF.
    Journal of neurochemistry, 2008, Volume: 106, Issue:1

    Topics: Adult; Aged; Aged, 80 and over; Amination; Biogenic Polyamines; Biomarkers; Brain; Female; Glutamine; Humans; Huntington Disease; Lysine; Male; Middle Aged; Neurochemistry; Protein Processing, Post-Translational; Putrescine; Spermidine; Transglutaminases; Up-Regulation

2008
Glutamine, glutamine synthetase and Huntington's disease.
    Lancet (London, England), 1981, Jun-27, Volume: 1, Issue:8235

    Topics: Glutamate-Ammonia Ligase; Glutamine; Humans; Huntington Disease

1981
Huntington's disease. Glutamate and aspartate metabolism in blood platelets.
    Journal of the neurological sciences, 1982, Volume: 53, Issue:3

    Topics: Adult; Asparagine; Aspartic Acid; Blood Platelets; Female; Glutamates; Glutamic Acid; Glutamine; Humans; Huntington Disease; Kinetics; Male; Middle Aged

1982
Glutamine synthetase and fructose-1, 6-diphosphatase activity in the putamen of control and Huntington's disease brain post mortem.
    Life sciences, 1983, Apr-25, Volume: 32, Issue:17

    Topics: Fructose-Bisphosphatase; Fructosephosphates; Glutamate-Ammonia Ligase; Glutamine; Humans; Huntington Disease; Putamen

1983
Cultured fibroblasts in Huntington's disease. I. Effects of L-glutamic acid.
    Archives of neurology, 1983, Volume: 40, Issue:1

    Topics: Adult; Cell Survival; Cells, Cultured; Fibroblasts; Glutamates; Glutamine; Humans; Huntington Disease; Middle Aged; Skin

1983
L-Glutamate toxicity in Huntington's disease fibroblasts.
    Biochemical and biophysical research communications, 1980, Jul-31, Volume: 95, Issue:2

    Topics: Biological Transport; Cell Survival; Cells, Cultured; Fibroblasts; Glutamates; Glutamine; Humans; Huntington Disease

1980
Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias.
    Nature, 1995, Nov-23, Volume: 378, Issue:6555

    Topics: Adult; Antibodies, Monoclonal; Ataxin-1; Ataxins; Blotting, Western; Cell Line; Cerebellar Ataxia; DNA-Binding Proteins; Female; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Male; Nerve Tissue Proteins; Nuclear Proteins; TATA-Box Binding Protein; Transcription Factors

1995
Expression of the Huntington's disease (IT15) protein product in HD patients.
    Human molecular genetics, 1995, Volume: 4, Issue:8

    Topics: Aged; Alleles; Brain; Case-Control Studies; Caudate Nucleus; Cell Line; Cerebral Cortex; Gene Expression; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Immunohistochemistry; Middle Aged; Minisatellite Repeats; Nerve Tissue Proteins; Nuclear Proteins; Proteins; Tissue Distribution; Trinucleotide Repeats

1995
Huntington's disease: the neuroexcitotoxin aspartate is increased in platelets and decreased in plasma.
    Journal of the neurological sciences, 1994, Dec-01, Volume: 127, Issue:1

    Topics: Adult; Aspartic Acid; Blood Platelets; Blood Proteins; Chromatography, High Pressure Liquid; Female; Glutamic Acid; Glutamine; Humans; Huntington Disease; Male; Middle Aged; Phenylalanine; Plasma

1994
Evidence for glutamate excitotoxicity in Huntington's disease with proton magnetic resonance spectroscopy.
    Lancet (London, England), 1994, May-07, Volume: 343, Issue:8906

    Topics: Corpus Striatum; Creatine; Glutamine; Humans; Huntington Disease; Magnetic Resonance Spectroscopy

1994
Glutamate in Huntington's disease.
    Lancet (London, England), 1994, Jul-16, Volume: 344, Issue:8916

    Topics: Corpus Striatum; Glutamine; Humans; Huntington Disease; Magnetic Resonance Spectroscopy

1994
Triplet repeat genes raise questions.
    Nature, 1994, Apr-21, Volume: 368, Issue:6473

    Topics: Biological Evolution; Glutamine; Humans; Huntington Disease; Repetitive Sequences, Nucleic Acid

1994
Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases.
    Proceedings of the National Academy of Sciences of the United States of America, 1994, Jun-07, Volume: 91, Issue:12

    Topics: Amino Acid Sequence; Genes, Dominant; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Hydrogen Bonding; Molecular Sequence Data; Muscular Atrophy, Spinal; Nerve Tissue Proteins; Nuclear Proteins; Protein Binding; Protein Structure, Secondary; Receptors, Androgen; Repetitive Sequences, Nucleic Acid; Spinocerebellar Degenerations

1994
Glutamine repeats as polar zippers: their role in inherited neurodegenerative disease.
    Molecular medicine (Cambridge, Mass.), 1995, Volume: 1, Issue:7

    Topics: Glutamine; Huntington Disease; Models, Molecular; Nerve Tissue Proteins; Nuclear Proteins; Protein Structure, Secondary; Repetitive Sequences, Nucleic Acid

1995
Proton magnetic resonance spectroscopy in Huntington's disease: evidence in favour of the glutamate excitotoxic theory.
    Movement disorders : official journal of the Movement Disorder Society, 1996, Volume: 11, Issue:2

    Topics: Aspartic Acid; Cerebral Cortex; Corpus Striatum; Genetic Carrier Screening; Glutamic Acid; Glutamine; Humans; Huntington Disease; Magnetic Resonance Imaging; Nerve Degeneration; Reference Values; Synaptic Transmission

1996
Analysis of polyglutamine-coding repeats in the TATA-binding protein in different human populations and in patients with schizophrenia and bipolar affective disorder.
    American journal of medical genetics, 1996, Sep-20, Volume: 67, Issue:5

    Topics: Africa; Bipolar Disorder; Cerebellar Ataxia; DNA-Binding Proteins; England; Ethnicity; Glutamine; Humans; Huntington Disease; India; Muscular Atrophy, Spinal; Peptides; Polymorphism, Genetic; Racial Groups; Schizophrenia; TATA-Box Binding Protein; Transcription Factors; Trinucleotide Repeats

1996
Identification of an HD patient with a (CAG)180 repeat expansion and the propagation of highly expanded CAG repeats in lambda phage.
    Human genetics, 1997, Volume: 99, Issue:5

    Topics: Animals; Bacteriophage lambda; Base Sequence; Cloning, Molecular; DNA Primers; Escherichia coli; Genetic Vectors; Genomic Library; Glutamine; Humans; Huntington Disease; Mice; Mice, Transgenic; Polymerase Chain Reaction; Trinucleotide Repeats

1997
Cloning and expression of the rat atrophin-I (DRPLA disease gene) homologue.
    Neurobiology of disease, 1995, Volume: 2, Issue:3

    Topics: Animals; Base Sequence; Blotting, Northern; Brain Diseases; Cloning, Molecular; DNA, Complementary; Gene Expression; Glutamine; Huntington Disease; In Situ Hybridization; Molecular Sequence Data; Point Mutation; Rats; Rats, Sprague-Dawley; RNA, Messenger; Trinucleotide Repeats

1995
Chemical synthesis of polar zipper peptides: motifs for potential protein association in inherited neurodegenerative diseases.
    Biomedical peptides, proteins & nucleic acids : structure, synthesis & biological activity, 1995, Volume: 1, Issue:4

    Topics: Amino Acid Sequence; Brain Diseases; Chromatography, High Pressure Liquid; Glutamine; Humans; Huntington Disease; Indicators and Reagents; Machado-Joseph Disease; Molecular Sequence Data; Muscular Dystrophies; Nerve Degeneration; Nerve Tissue Proteins; Peptides; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Spinocerebellar Degenerations

1995
Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats.
    Human molecular genetics, 1998, Volume: 7, Issue:5

    Topics: Animals; Cell Line; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Kidney; Nerve Tissue Proteins; Nuclear Proteins; PC12 Cells; Peptide Fragments; Rats; Repetitive Sequences, Nucleic Acid; Structure-Activity Relationship; Transfection

1998
Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.
    Human molecular genetics, 1998, Volume: 7, Issue:5

    Topics: Animals; Cell Line; Cell Nucleus; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Kidney; Mice; Nerve Tissue Proteins; Neuroblastoma; Nuclear Proteins; Peptide Fragments; Repetitive Sequences, Nucleic Acid; Staurosporine; Transfection; Tumor Cells, Cultured

1998
A fly's eye view of Huntington's disease.
    Nature, 1998, Oct-29, Volume: 395, Issue:6705

    Topics: Animals; Disease Models, Animal; Drosophila melanogaster; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Nerve Tissue Proteins; Nuclear Proteins; Photoreceptor Cells, Invertebrate; Recombinant Proteins

1998
SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates.
    Molecular cell, 1998, Volume: 2, Issue:4

    Topics: Adaptor Proteins, Signal Transducing; Animals; Brain Chemistry; Carrier Proteins; COS Cells; Exons; Gene Expression; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Nerve Tissue Proteins; Nuclear Proteins; Peptide Fragments; Precipitin Tests; Proline; Rabbits; Repetitive Sequences, Nucleic Acid; RNA, Messenger; src Homology Domains; Subcellular Fractions; Yeasts

1998
Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization.
    Nature medicine, 1999, Volume: 5, Issue:10

    Topics: Adolescent; Adult; Apoptosis; Caspase 3; Caspases; Cyanides; Enzyme Activation; Glutamine; Hematopoietic Stem Cells; Humans; Huntington Disease; Lymphocytes; Mitochondria; Staurosporine; Trinucleotide Repeats

1999
Preparation of human cDNas encoding expanded polyglutamine repeats.
    Neuroscience letters, 1999, Nov-12, Volume: 275, Issue:2

    Topics: DNA, Complementary; Glutamine; Humans; Huntington Disease; Myoclonic Epilepsies, Progressive; Polymerase Chain Reaction; Trinucleotide Repeat Expansion; Trinucleotide Repeats

1999
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice.
    Human molecular genetics, 2000, Mar-01, Volume: 9, Issue:4

    Topics: Animals; Cell Nucleus; Corpus Striatum; Cytoplasm; Glutamine; Huntingtin Protein; Huntington Disease; Immune Sera; Inclusion Bodies; Mice; Mice, Mutant Strains; Mutagenesis, Insertional; Nerve Tissue Proteins; Neurons; Nuclear Proteins; Peptides; Phenotype; Solubility

2000
Nonlinear decrease over time in N-acetyl aspartate levels in the absence of neuronal loss and increases in glutamine and glucose in transgenic Huntington's disease mice.
    Journal of neurochemistry, 2000, Volume: 74, Issue:5

    Topics: Animals; Aspartic Acid; Blood Glucose; Brain; Glucose; Glutamine; Humans; Huntington Disease; Magnetic Resonance Spectroscopy; Mice; Mice, Transgenic; Neurons; Nonlinear Dynamics; Time Factors

2000
Huntington's disease. In reverse gear.
    Nature, 2000, Apr-27, Volume: 404, Issue:6781

    Topics: Animals; Anti-Bacterial Agents; Disease Models, Animal; Gene Expression Regulation; Glutamine; Humans; Huntingtin Protein; Huntington Disease; Mice; Nerve Tissue Proteins; Nuclear Proteins; Trinucleotide Repeats

2000
The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis.
    Proceedings of the National Academy of Sciences of the United States of America, 2001, Feb-13, Volume: 98, Issue:4

    Topics: Age of Onset; Alanine; Animals; Brain; Caenorhabditis elegans; Dinucleotide Repeats; Glutamine; Helminth Proteins; Huntingtin Protein; Huntington Disease; Nerve Tissue Proteins; Nuclear Proteins; Recombinant Fusion Proteins; Trans-Activators; Transcriptional Elongation Factors

2001
Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats.
    Nature, 2001, Jul-12, Volume: 412, Issue:6843

    Topics: Age of Onset; Animals; Cell Death; Cell Nucleus; Glutamine; Humans; Huntington Disease; Neurodegenerative Diseases; Neurons; Repetitive Sequences, Amino Acid

2001
Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila.
    Nature, 2001, Oct-18, Volume: 413, Issue:6857

    Topics: Acetylation; Acetyltransferases; Animals; Animals, Genetically Modified; CREB-Binding Protein; Disease Models, Animal; Drosophila; Drosophila Proteins; E1A-Associated p300 Protein; Enzyme Inhibitors; Gene Expression Regulation; Glutamine; Glutathione Transferase; Histone Acetyltransferases; Histone Deacetylase Inhibitors; Histone Deacetylases; Histones; Huntingtin Protein; Huntington Disease; Nerve Degeneration; Nerve Tissue Proteins; Neurodegenerative Diseases; Nuclear Proteins; PC12 Cells; Peptides; Protein Structure, Tertiary; Rats; Repetitive Sequences, Amino Acid; Repressor Proteins; Saccharomyces cerevisiae Proteins; Sin3 Histone Deacetylase and Corepressor Complex; Trans-Activators

2001
Expression of full-length polyglutamine-expanded Huntingtin disrupts growth factor receptor signaling in rat pheochromocytoma (PC12) cells.
    The Journal of biological chemistry, 2002, Feb-22, Volume: 277, Issue:8

    Topics: Adrenal Gland Neoplasms; Animals; Enzyme Activation; Epidermal Growth Factor; ErbB Receptors; Glutamine; Humans; Huntingtin Protein; Huntington Disease; JNK Mitogen-Activated Protein Kinases; Mitogen-Activated Protein Kinases; Mutation; Nerve Growth Factor; Nerve Tissue Proteins; Nuclear Proteins; PC12 Cells; Peptides; Pheochromocytoma; Protein Serine-Threonine Kinases; Proto-Oncogene Proteins; Proto-Oncogene Proteins c-akt; Rats; Receptors, Growth Factor; Receptors, Nerve Growth Factor; Signal Transduction; Trinucleotide Repeat Expansion

2002
Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice.
    Human molecular genetics, 2002, Mar-15, Volume: 11, Issue:6

    Topics: Animals; Caspase 1; Cell Nucleus; Corpus Striatum; Gait; Genes, Dominant; Glutamine; Huntingtin Protein; Huntington Disease; Immunologic Factors; Mice; Mice, Knockout; Mice, Transgenic; Mutagenesis, Insertional; Nerve Tissue Proteins; Neurodegenerative Diseases; Nuclear Proteins; Phenotype; Time Factors; Trinucleotide Repeats

2002
Amyloid-like features of polyglutamine aggregates and their assembly kinetics.
    Biochemistry, 2002, Jun-11, Volume: 41, Issue:23

    Topics: Amyloid beta-Peptides; Antibodies, Monoclonal; Benzothiazoles; Binding Sites, Antibody; Fluorescent Dyes; Freezing; Glutamine; Hot Temperature; Humans; Huntington Disease; Kinetics; Microscopy, Electron; Peptides; Protein Binding; Solubility; Thiazoles

2002
Glucosamine dependence of Huntington's chorea fibroblasts in culture.
    Biochemical and biophysical research communications, 1976, May-23, Volume: 76, Issue:2

    Topics: Asparagine; Fibroblasts; Glucosamine; Glutamine; Glycine; Huntington Disease

1976
The cortical lesion of Huntington's disease: further neurochemical characterization, and reproduction of some of the histological and neurochemical features by N-methyl-D-aspartate lesions of rat cortex.
    Annals of neurology, 1992, Volume: 32, Issue:4

    Topics: Age Factors; Aged; Animals; Aspartic Acid; Brain Chemistry; Cholecystokinin; Female; gamma-Aminobutyric Acid; Glutamates; Glutamic Acid; Glutamine; Humans; Huntington Disease; Male; Middle Aged; N-Methylaspartate; Neurochemistry; Rats; Rats, Sprague-Dawley; Somatostatin; Substance P

1992
[Effect of administration of amino acids, especially of L-DOPA and -methyldopa, on the composition of cerebrospinal fluid in extrapyramidal syndromes. 3. Alterations of cerebrospinal fluid in patients with Huntington's chorea after -methyldopa or L-DOPA
    Zeitschrift fur Neurologie, 1973, Mar-16, Volume: 204, Issue:1

    Topics: Adult; Aged; Alanine Transaminase; Asparagine; Aspartic Acid; Child; Dihydroxyphenylalanine; Female; Glutamates; Glutamine; Glycine; Humans; Huntington Disease; Male; Methyldopa; Middle Aged; Phenylalanine; Pyruvates; Threonine

1973