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cystamine and Akinetic-Rigid Variant of Huntington Disease

cystamine has been researched along with Akinetic-Rigid Variant of Huntington Disease in 19 studies

Research Excerpts

ExcerptRelevanceReference
"Cystamine is beneficial to Huntington disease (HD) transgenic mice."7.73Treatment of YAC128 mice and their wild-type littermates with cystamine does not lead to its accumulation in plasma or brain: implications for the treatment of Huntington disease. ( Cooper, AJ; Hayden, MR; Jeitner, TM; Krasnikov, BF; Leavitt, BR; Pinto, JT; Thaler, HT; Van Raamsdonk, JM, 2005)
"Neurodegenerative disorders are a subset of disabling pathologies characterized, in part, by a progressive and specific loss of certain brain cell populations."6.47Potential of cystamine and cysteamine in the treatment of neurodegenerative diseases. ( Cicchetti, F; Gibrat, C, 2011)
"Cystamine treatment also ameliorated the striatal volume loss and striatal neuronal atrophy observed in these animals, but was unable to prevent motor dysfunction or the down-regulation of dopamine and cyclic adenosine monophsophate-regulated phosphoprotein (DARPP-32) expression in the striatum."5.33Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease. ( Bailey, CD; Hayden, MR; Johnson, GV; Leavitt, BR; Pearson, J; Rogers, DA; Van Raamsdonk, JM, 2005)
"Cystamine is a candidate drug; however, the mechanisms by which it operates remain unclear."5.33Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. ( Alberch, J; Borrell-Pagès, M; Brouillet, E; Bryson, EA; Canals, JM; Cheetham, ME; Cordelières, FP; Grange, G; Guillermier, M; Hantraye, P; Hirsch, E; Humbert, S; Néri, C; Parker, JA; Pineda, JR; Saudou, F, 2006)
"Cystamine is beneficial to Huntington disease (HD) transgenic mice."3.73Treatment of YAC128 mice and their wild-type littermates with cystamine does not lead to its accumulation in plasma or brain: implications for the treatment of Huntington disease. ( Cooper, AJ; Hayden, MR; Jeitner, TM; Krasnikov, BF; Leavitt, BR; Pinto, JT; Thaler, HT; Van Raamsdonk, JM, 2005)
" Treatment in R6/2 transgenic HD mice, using the transglutaminase inhibitor cystamine, significantly extended survival, improved body weight and motor performance, and delayed the neuropathological sequela."3.71Therapeutic effects of cystamine in a murine model of Huntington's disease. ( Beal, MF; Bogdanov, M; Cooper, AJ; Dedeoglu, A; Ferrante, RJ; Hersch, SM; Jeitner, TM; Kowall, NW; Kubilus, JK; Matson, SA; Matson, WR; Ratan, RR, 2002)
"Neurodegenerative disorders are a subset of disabling pathologies characterized, in part, by a progressive and specific loss of certain brain cell populations."2.47Potential of cystamine and cysteamine in the treatment of neurodegenerative diseases. ( Cicchetti, F; Gibrat, C, 2011)
"Cystamine is a byproduct of pantetheinase-catalyzed pantothenic acid recycling from pantetheine for biosynthesis of Coenzyme A (CoA), a ubiquitous and metabolically indispensable cofactor."2.43Post-translational disulfide modifications in cell signaling--role of inter-protein, intra-protein, S-glutathionyl, and S-cysteaminyl disulfide modifications in signal transmission. ( Chu, F; O'Brian, CA, 2005)
"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)
"Huntington's disease is a well-known genetic disorder associated with trinucleotide repeat expansions."1.37Therapeutic advances in the management of Huntington's disease. ( Abdulrahman, GO, 2011)
"Cystamine treatment also ameliorated the striatal volume loss and striatal neuronal atrophy observed in these animals, but was unable to prevent motor dysfunction or the down-regulation of dopamine and cyclic adenosine monophsophate-regulated phosphoprotein (DARPP-32) expression in the striatum."1.33Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease. ( Bailey, CD; Hayden, MR; Johnson, GV; Leavitt, BR; Pearson, J; Rogers, DA; Van Raamsdonk, JM, 2005)
"Cystamine is a candidate drug; however, the mechanisms by which it operates remain unclear."1.33Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. ( Alberch, J; Borrell-Pagès, M; Brouillet, E; Bryson, EA; Canals, JM; Cheetham, ME; Cordelières, FP; Grange, G; Guillermier, M; Hantraye, P; Hirsch, E; Humbert, S; Néri, C; Parker, JA; Pineda, JR; Saudou, F, 2006)
"In parts of the brain affected by Huntington's disease, the amount of the huntingtin with expanded polyglutamine is reduced and there appear huntingtin-containing polymers of larger molecular weight."1.30Transglutaminase action imitates Huntington's disease: selective polymerization of Huntingtin containing expanded polyglutamine. ( Djian, P; Green, H; Kahlem, P, 1998)

Research

Studies (19)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's1 (5.26)18.2507
2000's13 (68.42)29.6817
2010's5 (26.32)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Ribeiro, M1
Silva, AC1
Rodrigues, J1
Naia, L1
Rego, AC1
Bortvedt, SF1
McLear, JA1
Messer, A1
Ahern-Rindell, AJ1
Wolfgang, WJ1
Gibrat, C1
Cicchetti, F2
Sivananthan, SN1
Leavitt, BR3
Abdulrahman, GO1
Dedeoglu, A1
Kubilus, JK1
Jeitner, TM2
Matson, SA1
Bogdanov, M1
Kowall, NW1
Matson, WR1
Cooper, AJ2
Ratan, RR1
Beal, MF1
Hersch, SM2
Ferrante, RJ3
Lesort, M1
Lee, M1
Tucholski, J1
Johnson, GV3
Fox, JH1
Barber, DS1
Singh, B1
Zucker, B1
Swindell, MK1
Norflus, F1
Buzescu, R1
Chopra, R1
Kazantsev, A1
Zainelli, GM1
Dudek, NL1
Ross, CA1
Kim, SY1
Muma, NA1
Wang, X1
Sarkar, A1
Yu, M1
Zhu, A1
Jokivarsi, K1
Saint-Pierre, M1
Brownell, AL1
Bailey, CD2
Pinto, JT1
Van Raamsdonk, JM2
Hayden, MR2
Thaler, HT1
Krasnikov, BF1
O'Brian, CA1
Chu, F1
Pearson, J1
Rogers, DA1
Borrell-Pagès, M1
Canals, JM1
Cordelières, FP1
Parker, JA1
Pineda, JR1
Grange, G1
Bryson, EA1
Guillermier, M1
Hirsch, E1
Hantraye, P1
Cheetham, ME1
Néri, C1
Alberch, J1
Brouillet, E1
Saudou, F1
Humbert, S1
Frankish, H1
Ryu, H1
Lee, J1
Hagerty, SW1
Soh, BY1
McAlpin, SE1
Cormier, KA1
Smith, KM1
Kahlem, P1
Green, H1
Djian, P1
Karpuj, MV1
Becher, MW1
Springer, JE1
Chabas, D1
Youssef, S1
Pedotti, R1
Mitchell, D1
Steinman, L1

Reviews

2 reviews available for cystamine and Akinetic-Rigid Variant of Huntington Disease

ArticleYear
Potential of cystamine and cysteamine in the treatment of neurodegenerative diseases.
    Progress in neuro-psychopharmacology & biological psychiatry, 2011, Mar-30, Volume: 35, Issue:2

    Topics: Animals; Cystamine; Cysteamine; Disease Models, Animal; Enzyme Inhibitors; Humans; Huntington Diseas

2011
Post-translational disulfide modifications in cell signaling--role of inter-protein, intra-protein, S-glutathionyl, and S-cysteaminyl disulfide modifications in signal transmission.
    Free radical research, 2005, Volume: 39, Issue:5

    Topics: Animals; Cystamine; Disulfides; Glutathione; Humans; Huntington Disease; Mice; Oxidation-Reduction;

2005

Other Studies

17 other studies available for cystamine and Akinetic-Rigid Variant of Huntington Disease

ArticleYear
Oxidizing effects of exogenous stressors in Huntington's disease knock-in striatal cells--protective effect of cystamine and creatine.
    Toxicological sciences : an official journal of the Society of Toxicology, 2013, Volume: 136, Issue:2

    Topics: Corpus Striatum; Creatinine; Cystamine; Enzymes; Humans; Huntington Disease; Hydrogen Peroxide; Oxid

2013
Cystamine and intrabody co-treatment confers additional benefits in a fly model of Huntington's disease.
    Neurobiology of disease, 2010, Volume: 40, Issue:1

    Topics: Animals; Animals, Genetically Modified; Antibodies; Cystamine; Disease Models, Animal; Drosophila me

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
Therapeutic advances in the management of Huntington's disease.
    The Yale journal of biology and medicine, 2011, Volume: 84, Issue:3

    Topics: Cystamine; Humans; Huntington Disease; Peptides; Recombinant Fusion Proteins; RNA Interference; Sing

2011
Therapeutic effects of cystamine in a murine model of Huntington's disease.
    The Journal of neuroscience : the official journal of the Society for Neuroscience, 2002, Oct-15, Volume: 22, Issue:20

    Topics: Administration, Oral; Aged; Animals; Behavior, Animal; Biomarkers; Body Weight; Caudate Nucleus; Cys

2002
Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders.
    The Journal of biological chemistry, 2003, Feb-07, Volume: 278, Issue:6

    Topics: Caspase 3; Caspase Inhibitors; Caspases; Cystamine; Cysteine Proteinase Inhibitors; Enzyme Activatio

2003
Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation.
    Journal of neurochemistry, 2004, Volume: 91, Issue:2

    Topics: Animals; Antioxidants; Brain; Buthionine Sulfoximine; Cystamine; Cysteine; Cytoprotection; Disease M

2004
Mutant huntingtin protein: a substrate for transglutaminase 1, 2, and 3.
    Journal of neuropathology and experimental neurology, 2005, Volume: 64, Issue:1

    Topics: Aged; Aged, 80 and over; Alternative Splicing; Brain; Calcium-Binding Proteins; Cell Line; Cell Surv

2005
Cerebral PET imaging and histological evidence of transglutaminase inhibitor cystamine induced neuroprotection in transgenic R6/2 mouse model of Huntington's disease.
    Journal of the neurological sciences, 2005, Apr-15, Volume: 231, Issue:1-2

    Topics: Age Factors; Animals; Binding, Competitive; Body Weight; Brain; Brain Chemistry; Brain Mapping; Cere

2005
The protective effects of cystamine in the R6/2 Huntington's disease mouse involve mechanisms other than the inhibition of tissue transglutaminase.
    Neurobiology of aging, 2006, Volume: 27, Issue:6

    Topics: Age Factors; Age of Onset; Animals; Body Weight; Cystamine; Disease Models, Animal; Disease Progress

2006
Treatment of YAC128 mice and their wild-type littermates with cystamine does not lead to its accumulation in plasma or brain: implications for the treatment of Huntington disease.
    Journal of neurochemistry, 2005, Volume: 94, Issue:4

    Topics: Animals; Brain; Chromatography, High Pressure Liquid; Colorimetry; Cystamine; Cysteamine; Female; Gl

2005
Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease.
    Journal of neurochemistry, 2005, Volume: 95, Issue:1

    Topics: Administration, Oral; Animals; Corpus Striatum; Cystamine; Disease Models, Animal; Drug Administrati

2005
Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase.
    The Journal of clinical investigation, 2006, Volume: 116, Issue:5

    Topics: Aged; Animals; Brain; Brain-Derived Neurotrophic Factor; Caenorhabditis elegans; Cystamine; Cysteami

2006
Drug shows potential for treatment of Huntington's disease.
    The Lancet. Neurology, 2006, Volume: 5, Issue:6

    Topics: Brain-Derived Neurotrophic Factor; Cell Death; Cystamine; Cysteamine; Enzyme Inhibitors; Humans; Hun

2006
ESET/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's disease.
    Proceedings of the National Academy of Sciences of the United States of America, 2006, Dec-12, Volume: 103, Issue:50

    Topics: Aged; Animals; Cystamine; Female; Gene Expression; Histone-Lysine N-Methyltransferase; Histones; Hum

2006
Transglutaminase action imitates Huntington's disease: selective polymerization of Huntingtin containing expanded polyglutamine.
    Molecular cell, 1998, Volume: 1, Issue:4

    Topics: Adolescent; Adult; Age Factors; Cells, Cultured; Cerebral Cortex; Cystamine; Female; Humans; Hunting

1998
Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine.
    Nature medicine, 2002, Volume: 8, Issue:2

    Topics: Animals; Brain; Cystamine; Enzyme Inhibitors; Humans; Huntington Disease; Mice; Mice, Transgenic; Mo

2002