Page last updated: 2024-10-20

urea and Huntington Disease

urea has been researched along with Huntington Disease in 7 studies

pseudourea: clinical use; structure
isourea : A carboximidic acid that is the imidic acid tautomer of urea, H2NC(=NH)OH, and its hydrocarbyl derivatives.

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
" ARG2 protein is not reduced in the prodromal stage, though enzyme activity is reduced, indicating that altered Mn bioavailability as a cofactor leads to the deficient enzymatic activity."1.46Reduced bioavailable manganese causes striatal urea cycle pathology in Huntington's disease mouse model. ( Aschner, M; Bichell, TJV; Bowman, AB; Bradley, EM; Bryan, M; Colbran, RJ; Di Pardo, A; Dudek, K; Fisher, N; Halbesma, T; Holt, HK; Horning, K; Kwakye, GF; Maglione, V; Neely, MD; Osmand, A; Stubbs, AD; Tidball, AM; Tipps, KG; Uhouse, MA; Umashanker, P; Wegrzynowicz, M, 2017)
"We conclude that Huntington's disease is not associated with a generalised disturbance of quinolinic acid metabolism, however, a local hyperproduction of quinolinic acid cannot be excluded from our results."1.27Normal excretion of quinolinic acid in Huntington's disease. ( Brown, RR; Garnett, ES; Heyes, MP, 1985)

Research

Studies (7)

TimeframeStudies, this research(%)All Research%
pre-19901 (14.29)18.7374
1990's0 (0.00)18.2507
2000's2 (28.57)29.6817
2010's4 (57.14)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Handley, RR1
Reid, SJ1
Brauning, R1
Maclean, P1
Mears, ER1
Fourie, I1
Patassini, S1
Cooper, GJS1
Rudiger, SR1
McLaughlan, CJ1
Verma, PJ1
Gusella, JF1
MacDonald, ME1
Waldvogel, HJ1
Bawden, CS1
Faull, RLM1
Snell, RG1
Chen, CM3
Lin, YS1
Wu, YR2
Chen, P1
Tsai, FJ2
Yang, CL1
Tsao, YT1
Chang, W1
Hsieh, IS1
Chern, Y3
Soong, BW2
Bichell, TJV1
Wegrzynowicz, M1
Tipps, KG1
Bradley, EM1
Uhouse, MA1
Bryan, M1
Horning, K1
Fisher, N1
Dudek, K1
Halbesma, T1
Umashanker, P1
Stubbs, AD1
Holt, HK1
Kwakye, GF1
Tidball, AM1
Colbran, RJ1
Aschner, M1
Neely, MD1
Di Pardo, A1
Maglione, V1
Osmand, A1
Bowman, AB1
Chiang, MC2
Chen, HM2
Lai, HL1
Chen, HW1
Chou, SY1
England, JL1
Kaganovich, D1
Lee, YH1
Chang, HH1
Wu, YC1
Liu, CS1
Niu, DM1
Wu, JY1
Chen, YT1
Heyes, MP1
Garnett, ES1
Brown, RR1

Other Studies

7 other studies available for urea and Huntington Disease

ArticleYear
Brain urea increase is an early Huntington's disease pathogenic event observed in a prodromal transgenic sheep model and HD cases.
    Proceedings of the National Academy of Sciences of the United States of America, 2017, 12-26, Volume: 114, Issue:52

    Topics: Adult; Animals; Animals, Genetically Modified; Corpus Striatum; Disease Models, Animal; Female; Huma

2017
High Protein Diet and Huntington's Disease.
    PloS one, 2015, Volume: 10, Issue:5

    Topics: Adult; Citrulline; Dietary Proteins; Disease Progression; Female; Humans; Huntington Disease; Male;

2015
Reduced bioavailable manganese causes striatal urea cycle pathology in Huntington's disease mouse model.
    Biochimica et biophysica acta. Molecular basis of disease, 2017, Volume: 1863, Issue:6

    Topics: Animals; Arginase; Corpus Striatum; Disease Models, Animal; Huntington Disease; Male; Manganese; Mic

2017
The A2A adenosine receptor rescues the urea cycle deficiency of Huntington's disease by enhancing the activity of the ubiquitin-proteasome system.
    Human molecular genetics, 2009, Aug-15, Volume: 18, Issue:16

    Topics: Adenosine; Adenosine A2 Receptor Antagonists; Animals; Cell Line; Disease Models, Animal; Female; Hu

2009
Polyglutamine shows a urea-like affinity for unfolded cytosolic protein.
    FEBS letters, 2011, Jan-21, Volume: 585, Issue:2

    Topics: Animals; Cell Line; Cytosol; Humans; Huntington Disease; Microscopy, Confocal; Peptides; Protein Den

2011
Dysregulation of C/EBPalpha by mutant Huntingtin causes the urea cycle deficiency in Huntington's disease.
    Human molecular genetics, 2007, Mar-01, Volume: 16, Issue:5

    Topics: Animals; Brain-Derived Neurotrophic Factor; CCAAT-Enhancer-Binding Protein-alpha; Citrulline; Diet;

2007
Normal excretion of quinolinic acid in Huntington's disease.
    Life sciences, 1985, Nov-11, Volume: 37, Issue:19

    Topics: Adolescent; Adult; Creatinine; Female; Humans; Huntington Disease; Male; Middle Aged; Pyridines; Qui

1985