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malonic acid and Huntington Disease

malonic acid has been researched along with Huntington Disease in 18 studies

malonic acid : An alpha,omega-dicarboxylic acid in which the two carboxy groups are separated by a single methylene group.
dicarboxylic acid : Any carboxylic acid containing two carboxy groups.

Huntington Disease: A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)

Research Excerpts

ExcerptRelevanceReference
"Malonic acid is a reversible inhibitor of mitochondrial enzyme complex-II, induces energy crisis and free radical generation."1.72Protective effect of 3-n-butylphthalide against intrastriatal injection of malonic acid-induced neurotoxicity and biochemical alteration in rats. ( Yuan, C; Zhao, Y; Zheng, L, 2022)
"Malonic acid (MA) is a reversible inhibitor of succinate dehydrogenase (SDH) which induces mitochondrial dysfunction followed by secondary excitotoxicity and apoptosis due to generation of reactive oxygen species."1.39Synergistical neuroprotection of rofecoxib and statins against malonic acid induced Huntington's disease like symptoms and related cognitive dysfunction in rats. ( Kalonia, H; Kumar, A; Mishra, J; Sharma, N, 2013)
"Cystamine treatment of various genetic models of HD demonstrated protection against neurodegeneration and/or improvement in behavior."1.37Cystamine and ethyl-eicosapentaenoic acid treatment fail to prevent malonate-induced striatal toxicity in mice. ( Leavitt, BR; Sivananthan, SN, 2011)
"Transgenic Huntington's disease (HD) mice, expressing exon 1 of the HD gene with an expanded CAG repeat, are totally resistant to striatal lesion induced by excessive NMDA receptor activation."1.31Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length. ( Bates, GP; Brundin, P; Castilho, RF; Hansson, O; Korhonen, L; Lindholm, D, 2001)
"The gene defect in Huntington's disease (HD) may result in an impairment of energy metabolism."1.30Neuroprotective effects of creatine and cyclocreatine in animal models of Huntington's disease. ( Beal, MF; Ferrante, RJ; Jenkins, BG; Kaddurah-Daouk, R; Matthews, RT; Rosen, BR; Yang, L, 1998)

Research

Studies (18)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's1 (5.56)18.2507
2000's11 (61.11)29.6817
2010's4 (22.22)24.3611
2020's2 (11.11)2.80

Authors

AuthorsStudies
Yang, X1
Zhang, H1
Qu, T1
Wang, Y1
Zhong, Y1
Yan, Y1
Ji, X1
Chi, T1
Liu, P1
Zou, L1
Yuan, C1
Zheng, L1
Zhao, Y1
Kumar, A2
Sharma, N1
Mishra, J1
Kalonia, H2
Sagredo, O2
González, S1
Aroyo, I1
Pazos, MR1
Benito, C1
Lastres-Becker, I2
Romero, JP1
Tolón, RM1
Mechoulam, R1
Brouillet, E2
Romero, J1
Fernández-Ruiz, J4
Kumar, P1
Sivananthan, SN1
Leavitt, BR1
Valdeolivas, S1
Satta, V1
Pertwee, RG1
Petersén, A1
Puschban, Z1
Lotharius, J1
NicNiocaill, B1
Wiekop, P1
O'Connor, WT1
Brundin, P2
Bizat, N1
Boyer, F1
Hantraye, P1
Alfinito, PD1
Wang, SP1
Manzino, L1
Rijhsinghani, S1
Zeevalk, GD1
Sonsalla, PK1
Murphy, RC1
Messer, A1
Klivenyi, P3
Starkov, AA1
Calingasan, NY1
Gardian, G1
Browne, SE1
Yang, L2
Bubber, P1
Gibson, GE1
Patel, MS1
Beal, MF4
de Lago, E1
Ortega-Gutiérrez, S1
Cabranes, A1
Pryce, G1
Baker, D1
López-Rodríguez, M1
Ramos, JA1
Li, J1
Calkins, MJ1
Johnson, DA1
Johnson, JA1
Matthews, RT1
Jenkins, BG1
Ferrante, RJ3
Rosen, BR1
Kaddurah-Daouk, R1
Andreassen, OA2
Dedeoglu, A2
Mueller, G1
Lancelot, E1
Bogdanov, M1
Andersen, JK1
Jiang, D1
Hughes, DB1
Ona, VO1
Friedlander, RM1
Hansson, O1
Castilho, RF1
Korhonen, L1
Lindholm, D1
Bates, GP1

Reviews

1 review available for malonic acid and Huntington Disease

ArticleYear
Role of Nrf2-dependent ARE-driven antioxidant pathway in neuroprotection.
    Methods in molecular biology (Clifton, N.J.), 2007, Volume: 399

    Topics: Animals; Antioxidants; Cell Death; Corpus Striatum; Disease Models, Animal; Electron Transport Compl

2007

Other Studies

17 other studies available for malonic acid and Huntington Disease

ArticleYear
Tolfenamic acid inhibits ROS-generating oxidase Nox1-regulated p53 activity in intrastriatal injection of malonic acid rats.
    The journal of physiological sciences : JPS, 2022, Jul-18, Volume: 72, Issue:1

    Topics: Animals; Apoptosis; Huntington Disease; Malonates; Mice; Oxidoreductases; Rats; Reactive Oxygen Spec

2022
Protective effect of 3-n-butylphthalide against intrastriatal injection of malonic acid-induced neurotoxicity and biochemical alteration in rats.
    Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie, 2022, Volume: 155

    Topics: Animals; beta Catenin; Huntington Disease; Nerve Growth Factors; Neuroprotective Agents; Neurotoxici

2022
Synergistical neuroprotection of rofecoxib and statins against malonic acid induced Huntington's disease like symptoms and related cognitive dysfunction in rats.
    European journal of pharmacology, 2013, Jun-05, Volume: 709, Issue:1-3

    Topics: Animals; Atorvastatin; Behavior, Animal; Brain; Cognition Disorders; Cyclooxygenase 2 Inhibitors; Cy

2013
Cannabinoid CB2 receptor agonists protect the striatum against malonate toxicity: relevance for Huntington's disease.
    Glia, 2009, Aug-15, Volume: 57, Issue:11

    Topics: Animals; Arachidonic Acids; Camphanes; Cannabinoids; Cell Death; Central Nervous System Agents; Corp

2009
Targeting oxidative stress attenuates malonic acid induced Huntington like behavioral and mitochondrial alterations in rats.
    European journal of pharmacology, 2010, May-25, Volume: 634, Issue:1-3

    Topics: Animals; Body Weight; Disease Models, Animal; Drug Delivery Systems; Huntington Disease; Lipid Perox

2010
Cystamine and ethyl-eicosapentaenoic acid treatment fail to prevent malonate-induced striatal toxicity in mice.
    Neurobiology of aging, 2011, Volume: 32, Issue:12

    Topics: Animals; Corpus Striatum; Cystamine; Disease Models, Animal; Eicosapentaenoic Acid; Huntington Disea

2011
Sativex-like combination of phytocannabinoids is neuroprotective in malonate-lesioned rats, an inflammatory model of Huntington's disease: role of CB1 and CB2 receptors.
    ACS chemical neuroscience, 2012, May-16, Volume: 3, Issue:5

    Topics: Animals; Cannabidiol; Cannabinoids; Disease Models, Animal; Dronabinol; Drug Combinations; Drug Ther

2012
Evidence for dysfunction of the nigrostriatal pathway in the R6/1 line of transgenic Huntington's disease mice.
    Neurobiology of disease, 2002, Volume: 11, Issue:1

    Topics: Animals; Cell Count; Corpus Striatum; Disease Models, Animal; Dopamine; Female; Fluorescent Dyes; Hu

2002
Effects of cannabinoids in the rat model of Huntington's disease generated by an intrastriatal injection of malonate.
    Neuroreport, 2003, May-06, Volume: 14, Issue:6

    Topics: Animals; Cannabinoid Receptor Modulators; Cannabinoids; Corpus Striatum; Disease Models, Animal; Dro

2003
Adenosinergic protection of dopaminergic and GABAergic neurons against mitochondrial inhibition through receptors located in the substantia nigra and striatum, respectively.
    The Journal of neuroscience : the official journal of the Society for Neuroscience, 2003, Nov-26, Volume: 23, Issue:34

    Topics: Adenosine; Adenosine A1 Receptor Antagonists; Adenosine A2 Receptor Antagonists; Animals; Corpus Str

2003
A single-chain Fv intrabody provides functional protection against the effects of mutant protein in an organotypic slice culture model of Huntington's disease.
    Brain research. Molecular brain research, 2004, Feb-05, Volume: 121, Issue:1-2

    Topics: Animals; Animals, Newborn; Biolistics; Cell Aggregation; Cell Count; Cell Death; Corpus Striatum; Cu

2004
Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity.
    Journal of neurochemistry, 2004, Volume: 88, Issue:6

    Topics: 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine; Animals; Caudate Nucleus; Cell Count; Cerebral Cortex;

2004
UCM707, an inhibitor of the anandamide uptake, behaves as a symptom control agent in models of Huntington's disease and multiple sclerosis, but fails to delay/arrest the progression of different motor-related disorders.
    European neuropsychopharmacology : the journal of the European College of Neuropsychopharmacology, 2006, Volume: 16, Issue:1

    Topics: 3,4-Dihydroxyphenylacetic Acid; Analysis of Variance; Animals; Arachidonic Acids; Brain Chemistry; D

2006
Neuroprotective effects of creatine and cyclocreatine in animal models of Huntington's disease.
    The Journal of neuroscience : the official journal of the Society for Neuroscience, 1998, Jan-01, Volume: 18, Issue:1

    Topics: Adenosine Triphosphate; Animals; Antihypertensive Agents; Antineoplastic Agents; Creatine; Creatinin

1998
Mice deficient in cellular glutathione peroxidase show increased vulnerability to malonate, 3-nitropropionic acid, and 1-methyl-4-phenyl-1,2,5,6-tetrahydropyridine.
    The Journal of neuroscience : the official journal of the Society for Neuroscience, 2000, Jan-01, Volume: 20, Issue:1

    Topics: 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine; 3,4-Dihydroxyphenylacetic Acid; Animals; Brain Chemist

2000
Malonate and 3-nitropropionic acid neurotoxicity are reduced in transgenic mice expressing a caspase-1 dominant-negative mutant.
    Journal of neurochemistry, 2000, Volume: 75, Issue:2

    Topics: Animals; Brain; Caspase 1; Crosses, Genetic; Disease Models, Animal; Female; Huntington Disease; Mal

2000
Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length.
    Journal of neurochemistry, 2001, Volume: 78, Issue:4

    Topics: Aging; Amino Acid Chloromethyl Ketones; Animals; bcl-X Protein; Blood Glucose; Cell Death; Corpus St

2001