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The IGF-1/Akt Pathway Is Neuroprotective in Huntington's Disease and Involves Huntingtin Phosphorylation by Akt

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Neuroprotective Effects of Phenylbutyrate in the N171-82Q Transgenic Mouse Model of Huntington's Disease

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Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2′dG

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Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease

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A randomized sequential trial of creatine in amyotrophic lateral sclerosis

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Mutant Huntingtin Causes Context-Dependent Neurodegeneration in Mice with Huntington's Disease

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Mismatch repair gene Msh2 modifies the timing of early disease in HdhQ111 striatum

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Combination therapy with Coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson’s and Huntington’s Diseases

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Experimental therapeutics in transgenic mouse models of Huntington's disease

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Energy deficit in Huntington disease: why it matters

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Excitotoxic Neuronal Death and the Pathogenesis of Huntington's Disease

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Mice lacking alpha-synuclein are resistant to mitochondrial toxins

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The use of the R6 transgenic mouse models of Huntington’s disease in attempts to develop novel therapeutic strategies

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Health implications of creatine: can oral creatine supplementation protect against neurological and atherosclerotic disease?

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Synthesis and transport of creatine in the CNS: Importance for cerebral functions

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Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease

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Highly resolved in vivo 1H NMR spectroscopy of the mouse brain at 9.4 T

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Neuroprotective effects of creatine

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Proteomic Analysis of Protein Expression and Oxidative Modification in R6/2 Transgenic Mice

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Exendin-4 Improves Glycemic Control, Ameliorates Brain and Pancreatic Pathologies, and Extends Survival in a Mouse Model of Huntington's Disease

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Creatine and Its Potential Therapeutic Value for Targeting Cellular Energy Impairment in Neurodegenerative Diseases

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Cystamine Inhibits Caspase Activity

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Increased Susceptibility of Striatal Mitochondria to Calcium-Induced Permeability Transition

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Mitochondria and Huntington's Disease Pathogenesis: Insight from Genetic and Chemical Models

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Phosphorylation of profilin by ROCK1 regulates polyglutamine aggregation

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Minocycline and doxycycline are not beneficial in a model of Huntington's disease

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Triterpenoids CDDO-ethyl amide and CDDO-trifluoroethyl amide improve the behavioral phenotype and brain pathology in a transgenic mouse model of Huntington's disease

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Creatine for treating muscle disorders

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Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice

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The mitochondrial permeability transition in neurologic disease

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Evidence of Oxidant Damage in Huntington's Disease: Translational Strategies Using Antioxidants

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Redox Proteomics in Selected Neurodegenerative Disorders: From Its Infancy to Future Applications

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Metal‐Catalyzed Disruption of Membrane Protein and Lipid Signaling in the Pathogenesis of Neurodegenerative Disorders

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Creatine supplementation in Huntington’s disease

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Mitochondrial structural and functional dynamics in Huntington's disease

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Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease

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Antioxidants in Huntington's disease

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The mitochondrial cocktail: Rationale for combined nutraceutical therapy in mitochondrial cytopathies

Tarnopolsky, M. A. (2008) · Advanced Drug Delivery Reviews · doi:10.1016/j.addr.2008.05.001 · Cited by 161

The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

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Gene expression in Huntington's disease skeletal muscle: a potential biomarker

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Assessment of the nutrition status of patients with Huntington's disease

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Mitochondrial contact sites: their role in energy metabolism and apoptosis

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Role of oxidative DNA damage in mitochondrial dysfunction and Huntington’s disease pathogenesis

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Mitochondria targeted therapeutic approaches in Parkinson's and Huntington's diseases

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Induction of neostriatal neurogenesis slows disease progression in a transgenic murine model of Huntington disease

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Cardiac dysfunction in the R6/2 mouse model of Huntington’s disease

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Pharmacokinetics of the Dietary Supplement Creatine

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Creatine supplementation lowers brain glutamate levels in Huntington?s disease

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Pathophysiology of Huntingtonʼs disease: from huntingtin functions to potential treatments

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Application of MRS to mouse models of neurodegenerative illness

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Huntington's Disease of the Endocrine Pancreas: Insulin Deficiency and Diabetes Mellitus due to Impaired Insulin Gene Expression

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Skeletal muscle properties in a transgenic mouse model for amyotrophic lateral sclerosis: effects of creatine treatment

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Mass spectrometry and redox proteomics: Applications in disease

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Creatine improves health and survival of mice

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A small-molecule therapeutic lead for Huntington's disease: Preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse

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Environmental factors as modulators of neurodegeneration: Insights from gene–environment interactions in Huntington's disease

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Huntington Disease and the Huntingtin Protein

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Metabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic Treatments

Tefera, T. W., & Borges, K. (2017) · Frontiers in Neuroscience · doi:10.3389/fnins.2016.00611 · Cited by 100

The role of oxidative stress in Huntington’s disease: Are antioxidants good therapeutic candidates?

Gil-Mohapel, J., Brocardo, P., & Christie, B. (2014) · Current Drug Targets · doi:10.2174/1389450115666140115113734 · Cited by 101

The CREST-E study of creatine for Huntington disease

Hersch, S. M., Schifitto, G., Oakes, D., Bredlau, A., Meyers, C. M., Nahin, R. L., Rosas, H. D., & For the Huntington Study Group CREST-E Investigators and Coordinators (2017) · Neurology · doi:10.1212/wnl.0000000000004209 · Cited by 102

Neurodegenerative disorders: Parkinson's disease and Huntington's disease

Hague, S. (2005) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2004.060186 · Cited by 103

Creatine for neuroprotection in neurodegenerative disease: end of story?

Bender, A., & Klopstock, T. (2016) · Amino Acids · doi:10.1007/s00726-015-2165-0 · Cited by 96

Reappraisal of metabolic dysfunction in neurodegeneration: Focus on mitochondrial function and calcium signaling

Jadiya, P., Garbincius, J. F., & Elrod, J. W. (2021) · Acta Neuropathologica Communications · doi:10.1186/s40478-021-01224-4 · Cited by 94

Beyond muscles: The untapped potential of creatine

Riesberg, L. A., Weed, S. A., McDonald, T. L., Eckerson, J. M., & Drescher, K. M. (2016) · International Immunopharmacology · doi:10.1016/j.intimp.2015.12.034 · Cited by 95

Modelling brain diseases in mice: the challenges of design and analysis

Watase, K., & Zoghbi, H. Y. (2003) · Nature Reviews Genetics · doi:10.1038/nrg1045 · Cited by 94

Protein oxidation in Huntington disease affects energy production and vitamin B6 metabolism

Sorolla, M. A., Colman, M. J. R., Tamarit, J., Ortega, Z., Lucas, J. J., Ferrer, I., Ros, J., & Cabiscol, E. (2010) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2010.05.016 · Cited by 95

Pharmacologic approaches to the treatment of Huntington's disease

Venuto, C. S., McGarry, A. J., Ma, Q., & Kieburtz, K. D. (2011) · Movement Disorders · doi:10.1002/mds.23953 · Cited by 93

Mitochondrial Inhibition and Oxidative Stress: Reciprocating Players in Neurodegeneration

Zeevalk, G. D., Bernard, L. P., Song, C., Gluck, M. J., & Ehrhart, J. (2005) · Antioxidants and Redox Signaling · doi:10.1089/ars.2005.7.1117 · Cited by 89

Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease

Jenkins, B. G., Andreassen, O. A., Dedeoglu, A., Leavitt, B. R., Hayden, M. R., Borchelt, D., Ross, C. A., Ferrante, R. J., & Beal, M. F. (2005) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2005.03411.x · Cited by 83

1H NMR based metabolomics of CSF and blood serum: A metabolic profile for a transgenic rat model of Huntington disease

Verwaest, K. A., Vu, T. N., Laukens, K., Clemens, L. E., Nguyen, H. P., Gasse, B. V., Martins, J. C., der Linden, A. V., & Dommisse, R. A. (2011) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2011.08.001 · Cited by 83

A review of creatine supplementation in age-related diseases: more than a supplement for athletes

Smith, R. N., Agharkar, A. S., & Gonzales, E. B. (2014) · F1000Research · doi:10.12688/f1000research.5218.1 · Cited by 82

Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington's Disease

Ferrante, R. J., Andreassen, O. A., Jenkins, B. G., Dedeoglu, A., Kuemmerle, S., Kubilus, J. K., Kaddurah-Daouk, R., Hersch, S. M., & Beal, M. F. (2000) · The Journal of Neuroscience · doi:10.1523/jneurosci.20-12-04389.2000 · Cited by 522

Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice

Dedeoglu, A., Kubilus, J. K., Yang, L., Ferrante, K. L., Hersch, S. M., Beal, M. F., & Ferrante, A. R. J. (2003) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2003.01706.x · Cited by 169

Creatine-supplemented diet extends Purkinje cell survival in spinocerebellar ataxia type 1 transgenic mice but does not prevent the ataxic phenotype

Kaemmerer, W. F., Rodrigues, C. M. P., Steer, C. J., & Low, W. C. (2001) · Neuroscience · doi:10.1016/s0306-4522(01)00017-3 · Cited by 37

A modified gas-liquid chromatographic procedure for the rapid determination of bile acids in biological fluids

Nair, P. P., & Garcia, C. (1969) · Analytical Biochemistry · doi:10.1016/0003-2697(69)90020-7 · Cited by 107

Creatine: prescription for bad genes and a hostile environment?

Mattson, M. P. (2000) · Trends in Neurosciences · doi:10.1016/s0166-2236(00)01697-0 · Cited by 6