aspartic acid has been researched along with Huntington Disease in 52 studies
Aspartic Acid: One of the non-essential amino acids commonly occurring in the L-form. It is found in animals and plants, especially in sugar cane and sugar beets. It may be a neurotransmitter.
aspartic acid : An alpha-amino acid that consists of succinic acid bearing a single alpha-amino substituent
L-aspartic acid : The L-enantiomer of aspartic acid.
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)
| Excerpt | Relevance | Reference |
|---|---|---|
| " We found that S421 phosphorylation mitigates neurodegeneration by increasing proteasome-dependent turnover of mHTT and reducing the presence of a toxic mHTT conformer." | 1.43 | Serine 421 regulates mutant huntingtin toxicity and clearance in mice. ( Daub, AC; Finkbeiner, S; Gu, X; Humbert, S; Kratter, IH; Lau, A; Masliah, E; Osmand, A; Saudou, F; Steffan, JS; Tsvetkov, AS; Weiberth, KF; Yang, XW; Zahed, H, 2016) |
| "The precise pathogenic mechanisms of Huntington's disease (HD) are unknown but can be tested in vivo using proton magnetic resonance spectroscopy ((1)H MRS) to measure neurochemical changes." | 1.40 | Neurochemical correlates of caudate atrophy in Huntington's disease. ( Aylward, EH; Conley, KE; Laurino, MY; Padowski, JM; Richards, TL; Samii, A; Weaver, KE, 2014) |
| "Huntington's disease is an autosomal dominant disease which presents with striatal and cortical degeneration causing involuntary movements, dementia and emotional changes." | 1.31 | Altered striatal amino acid neurotransmitter release monitored using microdialysis in R6/1 Huntington transgenic mice. ( Brundin, P; Hansson, O; Haraldsson, B; Nicniocaill, B; O'Connor, WT, 2001) |
| "In all, 71 grafts in 38 patients [24 Parkinson's disease (PD), 14 Huntington's disease (HD)] were examined, as well as 24 untreated PD and HD patients and 13 age-matched normal controls." | 1.30 | In vivo magnetic resonance spectroscopy of human fetal neural transplants. ( Blüml, S; Dubowitz, D; Hoang, TQ; Jacques, DB; Kopyov, OV; Lin, A; Ross, BD; Seymour, K; Tan, J, 1999) |
| "The gene responsible for Huntington's disease (HD) has been located, but its action and the pathophysiology of HD remain unclear." | 1.29 | Proton magnetic resonance spectroscopy in Huntington's disease: evidence in favour of the glutamate excitotoxic theory. ( Brooks, DJ; Bryant, DJ; Harding, AE; Marcus, CD; Sargentoni, J; Taylor-Robinson, SD; Weeks, RA, 1996) |
| "Huntington's disease is a progressive neurodegenerative disease in which the basal ganglia are preferentially affected." | 1.28 | 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. ( Beal, MF; Finn, SF; Kowall, NW; Mazurek, MF; Storey, E, 1992) |
| "Though NAA could play a direct role in Huntington's disease, it seems more likely that the changes observed reflect illness or death of neurons, and that it may be feasible to monitor the course of Huntington's disease from NAA determinations." | 1.28 | Decreased brain N-acetylaspartate in Huntington's disease. ( Dunlop, DS; Lajtha, A; Mc Hale, DM, 1992) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 10 (19.23) | 18.7374 |
| 1990's | 11 (21.15) | 18.2507 |
| 2000's | 18 (34.62) | 29.6817 |
| 2010's | 13 (25.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Estrada-Sánchez, AM | 1 |
| Castro, D | 1 |
| Portillo-Ortiz, K | 1 |
| Jang, K | 1 |
| Nedjat-Haiem, M | 1 |
| Levine, MS | 1 |
| Cepeda, C | 1 |
| Casseb, RF | 1 |
| D'Abreu, A | 1 |
| Ruocco, HH | 2 |
| Lopes-Cendes, I | 2 |
| Cendes, F | 2 |
| Castellano, G | 1 |
| Padowski, JM | 1 |
| Weaver, KE | 1 |
| Richards, TL | 1 |
| Laurino, MY | 1 |
| Samii, A | 1 |
| Aylward, EH | 1 |
| Conley, KE | 1 |
| Yager, JR | 1 |
| Gasparovic, C | 1 |
| Magnotta, VA | 1 |
| Adams, W | 1 |
| Fiedorowicz, J | 1 |
| Paulsen, J | 1 |
| Jorge, R | 1 |
| Beglinger, LJ | 1 |
| van den Bogaard, SJ | 1 |
| Dumas, EM | 1 |
| Teeuwisse, WM | 1 |
| Kan, HE | 1 |
| Webb, A | 1 |
| van Buchem, MA | 1 |
| Roos, RA | 2 |
| van der Grond, J | 1 |
| Sturrock, A | 2 |
| Laule, C | 2 |
| Wyper, K | 1 |
| Milner, RA | 1 |
| Decolongon, J | 2 |
| Dar Santos, R | 2 |
| Coleman, AJ | 2 |
| Carter, K | 1 |
| Creighton, S | 2 |
| Bechtel, N | 2 |
| Bohlen, S | 1 |
| Reilmann, R | 4 |
| Johnson, HJ | 1 |
| Hayden, MR | 2 |
| Tabrizi, SJ | 4 |
| Mackay, AL | 2 |
| Leavitt, BR | 2 |
| Kratter, IH | 1 |
| Zahed, H | 1 |
| Lau, A | 1 |
| Tsvetkov, AS | 1 |
| Daub, AC | 1 |
| Weiberth, KF | 1 |
| Gu, X | 2 |
| Saudou, F | 1 |
| Humbert, S | 1 |
| Yang, XW | 3 |
| Osmand, A | 2 |
| Steffan, JS | 2 |
| Masliah, E | 1 |
| Finkbeiner, S | 2 |
| Sawiak, SJ | 1 |
| Wood, NI | 1 |
| Morton, AJ | 1 |
| Reynolds, NC | 2 |
| Prost, RW | 2 |
| Mark, LP | 2 |
| Joseph, SA | 1 |
| Greiner, ER | 1 |
| Mishra, R | 1 |
| Kodali, R | 1 |
| Thompson, LM | 1 |
| Wetzel, R | 1 |
| Ruggieri, M | 1 |
| Serpino, C | 1 |
| Ceci, E | 1 |
| Sciruicchio, V | 1 |
| Franco, G | 1 |
| Pica, C | 1 |
| Trojano, M | 1 |
| Livrea, P | 1 |
| de Tommaso, M | 1 |
| Miller, BR | 1 |
| Dorner, JL | 1 |
| Bunner, KD | 1 |
| Gaither, TW | 1 |
| Klein, EL | 1 |
| Barton, SJ | 1 |
| Rebec, GV | 1 |
| Unschuld, PG | 1 |
| Edden, RA | 1 |
| Carass, A | 1 |
| Liu, X | 1 |
| Shanahan, M | 1 |
| Wang, X | 1 |
| Oishi, K | 1 |
| Brandt, J | 1 |
| Bassett, SS | 1 |
| Redgrave, GW | 1 |
| Margolis, RL | 1 |
| van Zijl, PC | 1 |
| Barker, PB | 1 |
| Ross, CA | 2 |
| Gafni, J | 1 |
| Papanikolaou, T | 1 |
| Degiacomo, F | 1 |
| Holcomb, J | 1 |
| Chen, S | 1 |
| Menalled, L | 1 |
| Kudwa, A | 1 |
| Fitzpatrick, J | 1 |
| Miller, S | 1 |
| Ramboz, S | 1 |
| Tuunanen, PI | 1 |
| Lehtimäki, KK | 1 |
| Park, L | 1 |
| Kwak, S | 1 |
| Howland, D | 1 |
| Park, H | 1 |
| Ellerby, LM | 1 |
| Blamire, AM | 2 |
| Manners, DN | 2 |
| Rajagopalan, B | 2 |
| Styles, P | 3 |
| Schapira, AH | 2 |
| Warner, TT | 2 |
| Schapiro, M | 1 |
| Cecil, KM | 1 |
| Doescher, J | 1 |
| Kiefer, AM | 1 |
| Jones, BV | 1 |
| Bender, A | 1 |
| Auer, DP | 1 |
| Merl, T | 1 |
| Saemann, P | 1 |
| Yassouridis, A | 1 |
| Bender, J | 1 |
| Weindl, A | 1 |
| Dose, M | 1 |
| Gasser, T | 1 |
| Klopstock, T | 1 |
| Martin, WR | 1 |
| Wieler, M | 1 |
| Hanstock, CC | 1 |
| Li, LM | 1 |
| van Oostrom, JC | 1 |
| Sijens, PE | 1 |
| Leenders, KL | 1 |
| Ross, BD | 2 |
| Mangano, RM | 2 |
| Schwarcz, R | 2 |
| Rolf, LH | 1 |
| Lange, HW | 1 |
| Jenkins, BG | 5 |
| Koroshetz, WJ | 1 |
| Beal, MF | 7 |
| Rosen, BR | 3 |
| Brouillet, E | 1 |
| Chen, YC | 1 |
| Storey, E | 2 |
| Schulz, JB | 1 |
| Kirschner, P | 1 |
| Taylor-Robinson, SD | 1 |
| Weeks, RA | 1 |
| Bryant, DJ | 1 |
| Sargentoni, J | 1 |
| Marcus, CD | 1 |
| Harding, AE | 1 |
| Brooks, DJ | 1 |
| Harms, L | 1 |
| Meierkord, H | 1 |
| Timm, G | 1 |
| Pfeiffer, L | 1 |
| Ludolph, AC | 1 |
| Passani, LA | 1 |
| Vonsattel, JP | 1 |
| Carter, RE | 1 |
| Coyle, JT | 1 |
| Hoang, TQ | 1 |
| Blüml, S | 1 |
| Dubowitz, D | 1 |
| Kopyov, OV | 1 |
| Jacques, DB | 1 |
| Lin, A | 1 |
| Seymour, K | 1 |
| Tan, J | 1 |
| Klivenyi, P | 1 |
| Kustermann, E | 1 |
| Andreassen, OA | 3 |
| Ferrante, RJ | 3 |
| Dedeoglu, A | 2 |
| Kuemmerle, S | 1 |
| Kubilus, JK | 1 |
| Kaddurah-Daouk, R | 1 |
| Hersch, SM | 2 |
| Storgaard, J | 1 |
| Kornblit, BT | 1 |
| Zimmer, J | 1 |
| Gramsbergen, JB | 1 |
| van Dellen, A | 1 |
| Welch, J | 1 |
| Dixon, RM | 1 |
| Cordery, P | 1 |
| York, D | 1 |
| Blakemore, C | 1 |
| Hannan, AJ | 1 |
| Nicniocaill, B | 1 |
| Haraldsson, B | 1 |
| Hansson, O | 1 |
| O'Connor, WT | 1 |
| Brundin, P | 1 |
| Ferrante, KL | 1 |
| Thomas, M | 1 |
| Friedlich, A | 1 |
| Browne, SE | 1 |
| Schilling, G | 1 |
| Borchelt, DR | 1 |
| Liévens, JC | 1 |
| Woodman, B | 1 |
| Mahal, A | 1 |
| Spasic-Boscovic, O | 1 |
| Samuel, D | 2 |
| Kerkerian-Le Goff, L | 1 |
| Bates, GP | 1 |
| Kowall, NW | 1 |
| Finn, SF | 2 |
| Mazurek, MF | 1 |
| Dunlop, DS | 1 |
| Mc Hale, DM | 1 |
| Lajtha, A | 1 |
| Blin, O | 1 |
| Guieu, R | 1 |
| Pouget, J | 1 |
| Nieoullon, A | 1 |
| Serratrice, G | 1 |
| Albin, RL | 1 |
| Young, AB | 1 |
| Penney, JB | 1 |
| Handelin, B | 1 |
| Balfour, R | 1 |
| Anderson, KD | 1 |
| Markel, DS | 1 |
| Tourtellotte, WW | 1 |
| Reiner, A | 1 |
| Swartz, KJ | 1 |
| Bird, ED | 1 |
| Martin, JB | 1 |
| Meldrum, B | 1 |
| May, PC | 1 |
| Gray, PN | 1 |
| Cross, AJ | 1 |
| Slater, P | 1 |
| Reynolds, GP | 1 |
| Koh, JY | 1 |
| Peters, S | 1 |
| Choi, DW | 1 |
| Stone, TW | 1 |
| Connick, JH | 1 |
| Winn, P | 1 |
| Hastings, MH | 1 |
| English, M | 1 |
| Bonilla, E | 1 |
| Prasad, AL | 1 |
| Arrieta, A | 1 |
| Simanyi, M | 1 |
| Gerstenbrand, F | 1 |
| Gründig, E | 1 |
| Schedl, R | 1 |
| Weiss, H | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Coenzyme Q10 in Huntington's Disease (HD)[NCT00608881] | Phase 3 | 609 participants (Actual) | Interventional | 2008-03-31 | Terminated (stopped due to Futility analysis failed to showed likelihoo of benefit of CoQ 2400 mg/day.) | ||
| Creatine Safety, Tolerability, & Efficacy in Huntington's Disease (CREST-E)[NCT00712426] | Phase 3 | 553 participants (Actual) | Interventional | 2009-09-30 | Terminated (stopped due to Results of an interim analysis showed that it was unlikely that creatine was effective in slowing loss of function in early symptomatic Huntington's Disease.) | ||
| Creatine Safety and Tolerability in Premanifest HD: PRECREST[NCT00592995] | Phase 2 | 64 participants (Actual) | Interventional | 2007-12-31 | Completed | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
The Unified Huntington's Disease Rating Scale (UHDRS) behavioral subscale assesses frequency and severity of psychiatric-related symptoms, including depressed mood, apathy, low self-esteem/guilt, suicidal thoughts, anxiety, irritable behavior, aggressive behavior, obsessional thinking, compulsive behavior, delusions, and hallucinations. A total score was calculated by summing up all the individual behavioral frequency items (range 0-56) with higher scores representing more severe behavioral impairment. (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | 1.39 |
| B - Placebo | 1.43 |
The Unified Huntington's Disease Rating Scale (UHDRS) behavioral subscale assesses frequency and severity of psychiatric-related symptoms, including depressed mood, apathy, low self-esteem/guilt, suicidal thoughts, anxiety, irritable behavior, aggressive behavior, obsessional thinking, compulsive behavior, delusions, and hallucinations. The total score is the sum of the product of the individual behavioral frequency and severity items (range 0-176) with higher scores representing more severe behavioral impairment. (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | 4.29 |
| B - Placebo | 5.06 |
"The functional assessment checklist includes 25 questions about common daily tasks. A score of 1 is given for each yes reply and a score of 0 is given for each no reply (scale range is 0-25). Higher scores indicate better functioning." (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | -7.93 |
| B - Placebo | -8.02 |
The independence scale assesses independence on a 0 to 100 scale with higher scores indicating better functioning. (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | -26.30 |
| B - Placebo | -24.86 |
Stroop Interference Test - color naming score is the total number of correct colors identified in 45 seconds and reflects processing speed. (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | -14.21 |
| B - Placebo | -14.51 |
Stroop Interference Test - interference score is the total number of correct items identified in 45 seconds and reflects an executive measure of inhibitory ability. (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | -7.57 |
| B - Placebo | -8.61 |
Stroop Interference Test - word reading score is the total number of correct words read in 45 seconds and reflects processing speed. (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | -15.25 |
| B - Placebo | -19.13 |
The SDMT assesses attention, visuoperceptual processing, working memory, and cognitive/psychomotor speed. The score is the number of correctly paired abstract symbols and specific numbers in 90 seconds with higher scores indicating better cognitive functioning. (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | -10.95 |
| B - Placebo | -11.36 |
TFC consists of five ordinally scaled items assessing a person's capacity with: (1) occupation; (2) financial affairs; (3) domestic responsibilities; (4) activities of daily living; and (5) independent living. Total score ranges from zero (worst) to 13 (best). (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | -4.53 |
| B - Placebo | -4.76 |
The motor section of the Unified Huntington's Disease Rating Scale (UHDRS) assesses motor features of Huntington disease with standardized ratings of oculomotor function, dysarthria, chorea, dystonia, gait, and postural stability. The total motor score is the sum of all the individual motor ratings, with higher scores (124) indicating more severe motor impairment than lower scores. The score ranges from 0 to 124. (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | 18.06 |
| B - Placebo | 19.18 |
The verbal fluency test is typically considered a measure of executive function. The score is the number of correct words produced across three 1-minute trials. (NCT00608881)
Timeframe: Baseline and Month 60
| Intervention | units on a scale (Least Squares Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | -5.07 |
| B - Placebo | -4.47 |
The primary outcome variable at the start of the trial was the change in TFC score from baseline to Month 60. The Data and Safety Monitoring Board recommended to the trial leadership that they reconsider how they accommodate missing data from subjects who die in their primary analysis of the change in TFC score. Based on these recommendations, the trial leadership changed the primary analysis to that of a joint rank approach. TFC consists of five ordinally scaled items assessing a person's capacity with: (1) occupation; (2) financial affairs; (3) domestic responsibilities; (4) activities of daily living; and (5) independent living. Total score ranges from zero (worst) to 13 (best). (NCT00608881)
Timeframe: 5 years
| Intervention | rank (Mean) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | 303.3 |
| B - Placebo | 306.7 |
(NCT00608881)
Timeframe: 5 years
| Intervention | participants completing study on drug (Number) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | 98 |
| B - Placebo | 108 |
TFC consists of five ordinally scaled items assessing a person's capacity with: (1) occupation; (2) financial affairs; (3) domestic responsibilities; (4) activities of daily living; and (5) independent living. Total score ranges from zero (worst) to 13 (best). (NCT00608881)
Timeframe: 5 years
| Intervention | days to event (Median) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | 917 |
| B - Placebo | 911 |
TFC consists of five ordinally scaled items assessing a person's capacity with: (1) occupation; (2) financial affairs; (3) domestic responsibilities; (4) activities of daily living; and (5) independent living. Total score ranges from zero (worst) to 13 (best). (NCT00608881)
Timeframe: 5 years
| Intervention | days to event (Median) |
|---|---|
| A - Coenzyme Q10 2400 mg/Day | 553 |
| B - Placebo | 549 |
2 reviews available for aspartic acid and Huntington Disease
| Article | Year |
|---|---|
Possible therapeutic applications of antagonists of excitatory amino acid neurotransmitters.
Topics: Alzheimer Disease; Animals; Aspartic Acid; Basal Ganglia Diseases; Brain; Brain Ischemia; Epilepsy; | 1985 |
Endogenous excitotoxic agents.
Topics: Animals; Aspartic Acid; Cell Survival; Central Nervous System; Dementia; Epilepsy; Glutamates; Gluta | 1987 |
3 trials available for aspartic acid and Huntington Disease
| Article | Year |
|---|---|
Creatine therapy for Huntington's disease: clinical and MRS findings in a 1-year pilot study.
Topics: Adenosine Triphosphate; Aspartic Acid; Brain; Choline; Creatine; Diarrhea; Dietary Supplements; Dose | 2003 |
Creatine supplementation lowers brain glutamate levels in Huntington's disease.
Topics: Administration, Oral; Adult; Aspartic Acid; Brain; Cerebral Cortex; Creatine; Down-Regulation; Energ | 2005 |
High-dose creatine therapy for Huntington disease: a 2-year clinical and MRS study.
Topics: Administration, Oral; Aspartic Acid; Body Weight; Brain; Creatine; Creatinine; Dose-Response Relatio | 2005 |
47 other studies available for aspartic acid and Huntington Disease
| Article | Year |
|---|---|
Complete but not partial inhibition of glutamate transporters exacerbates cortical excitability in the R6/2 mouse model of Huntington's disease.
Topics: Animals; Aspartic Acid; Benzopyrans; Cerebral Cortex; Disease Models, Animal; Excitatory Amino Acid | 2019 |
Thalamic metabolic abnormalities in patients with Huntington's disease measured by magnetic resonance spectroscopy.
Topics: Adolescent; Adult; Aged; Aspartic Acid; Case-Control Studies; Creatine; Deuterium; Dipeptides; Femal | 2013 |
Neurochemical correlates of caudate atrophy in Huntington's disease.
Topics: Adult; Aged; Aspartic Acid; Atrophy; Caudate Nucleus; Female; Glutamic Acid; Humans; Huntington Dise | 2014 |
Preliminary study of the association of white-matter metabolite concentrations with disease severity in patients with Huntington's disease.
Topics: Aspartic Acid; Humans; Huntington Disease; Magnetic Resonance Spectroscopy; Nerve Fibers, Myelinated | 2014 |
Longitudinal metabolite changes in Huntington's disease during disease onset.
Topics: Adult; Aspartic Acid; Caudate Nucleus; Creatinine; Female; Follow-Up Studies; Humans; Huntington Dis | 2014 |
A longitudinal study of magnetic resonance spectroscopy Huntington's disease biomarkers.
Topics: Adult; Analysis of Variance; Aspartic Acid; Biomarkers; Brain; Cross-Sectional Studies; Female; Huma | 2015 |
Serine 421 regulates mutant huntingtin toxicity and clearance in mice.
Topics: Alanine; Animals; Aspartic Acid; Behavior, Animal; Chromosomes, Artificial, Bacterial; Disease Model | 2016 |
Similar Progression of Morphological and Metabolic Phenotype in R6/2 Mice with Different CAG Repeats Revealed by In Vivo Magnetic Resonance Imaging and Spectroscopy.
Topics: Animals; Aspartic Acid; Brain; Disease Models, Animal; Disease Progression; Glutamic Acid; Humans; H | 2016 |
MR-spectroscopic findings in juvenile-onset Huntington's disease.
Topics: Adolescent; Aspartic Acid; Brain Mapping; Child; Child, Preschool; Creatine; Female; Glutamic Acid; | 2008 |
Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice.
Topics: Alanine; Amino Acid Sequence; Amino Acid Substitution; Amyloid; Animals; Aspartic Acid; Disease Mode | 2009 |
Magnetic resonance spectroscopy biomarkers in premanifest and early Huntington disease.
Topics: Adult; Aspartic Acid; Biomarkers; Cohort Studies; Early Diagnosis; Female; Humans; Huntington Diseas | 2010 |
Serum levels of N-acetylaspartate in Huntington's disease: preliminary results.
Topics: Adolescent; Adult; Aged; Aspartic Acid; Case-Control Studies; Female; Humans; Huntington Disease; Ma | 2012 |
Up-regulation of GLT1 reverses the deficit in cortically evoked striatal ascorbate efflux in the R6/2 mouse model of Huntington's disease.
Topics: Animals; Ascorbic Acid; Ascorbic Acid Deficiency; Aspartic Acid; Ceftriaxone; Cerebral Cortex; Corpu | 2012 |
Brain metabolite alterations and cognitive dysfunction in early Huntington's disease.
Topics: Adult; Analysis of Variance; Aspartic Acid; Brain; Cognition Disorders; Female; Glutamic Acid; Human | 2012 |
Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment.
Topics: Age Factors; Amino Acids; Animals; Aspartic Acid; Body Weight; Brain; Caspase 6; Cells, Cultured; Co | 2012 |
MR imaging and spectroscopy in juvenile Huntington disease.
Topics: Aspartic Acid; Brain; Caudate Nucleus; Child; Creatinine; Genetic Testing; Humans; Huntington Diseas | 2004 |
Heterogeneity in 1H-MRS profiles of presymptomatic and early manifest Huntington's disease.
Topics: Adult; Age of Onset; Aged; Aspartic Acid; Early Diagnosis; Female; Glutamic Acid; Glutamine; Humans; | 2005 |
Is brain lactate increased in Huntington's disease?
Topics: Adult; Aspartic Acid; Brain; Case-Control Studies; Choline; Creatine; Female; Humans; Huntington Dis | 2007 |
Evidence of thalamic dysfunction in Huntington disease by proton magnetic resonance spectroscopy.
Topics: Adult; Age of Onset; Aged; Analysis of Variance; Aspartic Acid; Creatine; Female; Humans; Huntington | 2007 |
1H magnetic resonance spectroscopy in preclinical Huntington disease.
Topics: Adult; Age Factors; Analysis of Variance; Aspartic Acid; Brain; Brain Mapping; Choline; Creatine; Fe | 2007 |
Re: Long-term fetal cell transplant in Huntington disease: stayin' alive.
Topics: Aspartic Acid; Cell Differentiation; Corpus Striatum; Fetal Tissue Transplantation; Graft Survival; | 2008 |
Platelet glutamate and aspartate uptake in Huntington's disease.
Topics: Aspartic Acid; Biological Transport; Blood Platelets; Glutamates; Glutamic Acid; Humans; Huntington | 1981 |
Huntington's disease. Glutamate and aspartate metabolism in blood platelets.
Topics: Adult; Asparagine; Aspartic Acid; Blood Platelets; Female; Glutamates; Glutamic Acid; Glutamine; Hum | 1982 |
Huntington's disease: the neuroexcitotoxin aspartate is increased in platelets and decreased in plasma.
Topics: Adult; Aspartic Acid; Blood Platelets; Blood Proteins; Chromatography, High Pressure Liquid; Female; | 1994 |
Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy.
Topics: Adult; Aspartic Acid; Basal Ganglia; Choline; Creatine; Energy Metabolism; Female; Humans; Huntingto | 1993 |
Non-invasive neurochemical analysis of focal excitotoxic lesions in models of neurodegenerative illness using spectroscopic imaging.
Topics: Aging; Animals; Aspartic Acid; Electron Transport Complex II; Energy Metabolism; Huntington Disease; | 1996 |
Proton magnetic resonance spectroscopy in Huntington's disease: evidence in favour of the glutamate excitotoxic theory.
Topics: Aspartic Acid; Cerebral Cortex; Corpus Striatum; Genetic Carrier Screening; Glutamic Acid; Glutamine | 1996 |
Decreased N-acetyl-aspartate/choline ratio and increased lactate in the frontal lobe of patients with Huntington's disease: a proton magnetic resonance spectroscopy study.
Topics: Adult; Aged; Aspartic Acid; Choline; Female; Frontal Lobe; Humans; Huntington Disease; Lactic Acid; | 1997 |
N-acetylaspartylglutamate, N-acetylaspartate, and N-acetylated alpha-linked acidic dipeptidase in human brain and their alterations in Huntington and Alzheimer's diseases.
Topics: Alzheimer Disease; Amino Acids; Antigens, Surface; Aspartic Acid; Brain; Case-Control Studies; Cell | 1997 |
In vivo magnetic resonance spectroscopy of human fetal neural transplants.
Topics: Adult; Aspartic Acid; Brain; Fetal Tissue Transplantation; Humans; Huntington Disease; Magnetic Reso | 1999 |
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.
Topics: Animals; Aspartic Acid; Blood Glucose; Brain; Glucose; Glutamine; Humans; Huntington Disease; Magnet | 2000 |
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
Topics: Animals; Animals, Genetically Modified; Aspartic Acid; Atrophy; Brain; Corpus Striatum; Creatine; Cr | 2000 |
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
Topics: Animals; Animals, Genetically Modified; Aspartic Acid; Atrophy; Brain; Corpus Striatum; Creatine; Cr | 2000 |
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
Topics: Animals; Animals, Genetically Modified; Aspartic Acid; Atrophy; Brain; Corpus Striatum; Creatine; Cr | 2000 |
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
Topics: Animals; Animals, Genetically Modified; Aspartic Acid; Atrophy; Brain; Corpus Striatum; Creatine; Cr | 2000 |
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
Topics: Animals; Animals, Genetically Modified; Aspartic Acid; Atrophy; Brain; Corpus Striatum; Creatine; Cr | 2000 |
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
Topics: Animals; Animals, Genetically Modified; Aspartic Acid; Atrophy; Brain; Corpus Striatum; Creatine; Cr | 2000 |
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
Topics: Animals; Animals, Genetically Modified; Aspartic Acid; Atrophy; Brain; Corpus Striatum; Creatine; Cr | 2000 |
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
Topics: Animals; Animals, Genetically Modified; Aspartic Acid; Atrophy; Brain; Corpus Striatum; Creatine; Cr | 2000 |
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
Topics: Animals; Animals, Genetically Modified; Aspartic Acid; Atrophy; Brain; Corpus Striatum; Creatine; Cr | 2000 |
3-Nitropropionic acid neurotoxicity in organotypic striatal and corticostriatal slice cultures is dependent on glucose and glutamate.
Topics: Animals; Aspartic Acid; Cells, Cultured; Cerebral Cortex; Corpus Striatum; Disease Models, Animal; D | 2000 |
N-Acetylaspartate and DARPP-32 levels decrease in the corpus striatum of Huntington's disease mice.
Topics: Animals; Aspartic Acid; Biomarkers; Calbindins; Choline; Corpus Striatum; Creatine; Dopamine and cAM | 2000 |
Altered striatal amino acid neurotransmitter release monitored using microdialysis in R6/1 Huntington transgenic mice.
Topics: Amino Acids; Animals; Aspartic Acid; Corpus Striatum; gamma-Aminobutyric Acid; Glutamic Acid; Huntin | 2001 |
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease.
Topics: Animals; Aspartic Acid; Blood Glucose; Brain Chemistry; Cell Survival; Creatinine; Disease Models, A | 2001 |
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease.
Topics: Animals; Aspartic Acid; Blood Glucose; Brain Chemistry; Cell Survival; Creatinine; Disease Models, A | 2001 |
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease.
Topics: Animals; Aspartic Acid; Blood Glucose; Brain Chemistry; Cell Survival; Creatinine; Disease Models, A | 2001 |
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease.
Topics: Animals; Aspartic Acid; Blood Glucose; Brain Chemistry; Cell Survival; Creatinine; Disease Models, A | 2001 |
Impaired glutamate uptake in the R6 Huntington's disease transgenic mice.
Topics: Amino Acid Transport System X-AG; Animals; Aspartic Acid; Astrocytes; Biological Transport; Carrier | 2001 |
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.
Topics: Age Factors; Aged; Animals; Aspartic Acid; Brain Chemistry; Cholecystokinin; Female; gamma-Aminobuty | 1992 |
Decreased brain N-acetylaspartate in Huntington's disease.
Topics: Aspartic Acid; Brain; Female; Humans; Huntington Disease; Male | 1992 |
[Familial amyotrophic lateral sclerosis associated with Huntington chorea with increased aspartate level in the cerebrospinal fluid].
Topics: Amino Acids; Amyotrophic Lateral Sclerosis; Aspartic Acid; Chromatography, High Pressure Liquid; Fem | 1992 |
Abnormalities of striatal projection neurons and N-methyl-D-aspartate receptors in presymptomatic Huntington's disease.
Topics: Adult; Aspartic Acid; Corpus Striatum; Female; Heterozygote; Humans; Huntington Disease; Immunohisto | 1990 |
Amino acid and neuropeptide neurotransmitters in Huntington's disease cerebellum.
Topics: Aged; Aspartic Acid; Cerebellum; Female; Humans; Huntington Disease; Male; Middle Aged; Neuropeptide | 1988 |
L-Homocysteic acid as an alternative cytotoxin for studying glutamate-induced cellular degeneration of Huntington's disease and normal skin fibroblasts.
Topics: Aspartic Acid; Cell Survival; Cells, Cultured; Cysteic Acid; Cysteine; Fibroblasts; Glutamates; Glut | 1985 |
Reduced high-affinity glutamate uptake sites in the brains of patients with Huntington's disease.
Topics: Aged; Aspartic Acid; Brain; Caudate Nucleus; Female; Frontal Lobe; Glutamates; Glutamic Acid; Hippoc | 1986 |
Neurons containing NADPH-diaphorase are selectively resistant to quinolinate toxicity.
Topics: Animals; Aspartic Acid; Glutamates; Glutamic Acid; Humans; Huntington Disease; Kainic Acid; Mice; N- | 1986 |
Huntington's disease: studies on brain free amino acids.
Topics: Adult; Aged; Aged, 80 and over; Amino Acids; Aspartic Acid; Brain; Female; gamma-Aminobutyric Acid; | 1988 |
[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
Topics: Adult; Aged; Alanine Transaminase; Asparagine; Aspartic Acid; Child; Dihydroxyphenylalanine; Female; | 1973 |