JCI - Citations to Energy deficit in Huntington disease: why it matters

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Energy deficit in Huntington disease: why it matters

Fanny Mochel, Ronald G. Haller

Fanny Mochel, Ronald G. Haller

Published February 1, 2011
Citation Information: J Clin Invest. 2011;121(2):493-499. https://doi.org/10.1172/JCI45691.

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Citations to this article (93)

Title and authors	Publication	Year
Associations Between Diabetes Mellitus and Neurodegenerative Diseases Szablewski L	International Journal of Molecular Sciences	2025
SIRT3 Activation a Promise in Drug Development? New Insights into SIRT3 Biology and Its Implications on the Drug Discovery Process. Lambona C, Zwergel C, Valente S, Mai A	Journal of Medicinal Chemistry	2024
Investigating the Dietary Intake Using the CyFFQ Semi-Quantitative Food Frequency Questionnaire in Cypriot Huntington’s Disease Patients Christodoulou CC, Demetriou CA, Philippou E, Papanicolaou EZ	Nutrients	2023
Targeting Sigma Receptors for the Treatment of Neurodegenerative and Neurodevelopmental Disorders. Malar DS, Thitilertdecha P, Ruckvongacheep KS, Brimson S, Tencomnao T, Brimson JM	CNS Drugs	2023
Small Molecule Pytren-4QMn Metal Complex Slows down Huntington’s Disease Progression in Male zQ175 Transgenic Mice Merino M, González S, Tronch MC, Sánchez-Sánchez AV, Clares MP, García-España A, García-España E, Mullor JL	International journal of molecular sciences	2023
Function and therapeutic value of astrocytes in neurological diseases H Lee, M Wheeler, F Quintana	Nature Reviews Drug Discovery	2022
An orally available, brain penetrant, small molecule lowers huntingtin levels by enhancing pseudoexon inclusion C Keller, Y Shin, A Monteys, N Renaud, M Beibel, N Teider, T Peters, T Faller, S St-Cyr, J Knehr, G Roma, A Reyes, M Hild, D Lukashev, D Theil, N Dales, J Cha, B Borowsky, R Dolmetsch, B Davidson, R Sivasankaran	Nature Communications	2022
The Reversible Carnitine Palmitoyltransferase 1 Inhibitor (Teglicar) Ameliorates the Neurodegenerative Phenotype in a Drosophila Huntington’s Disease Model by Acting on the Expression of Carnitine-Related Genes Bertapelle C, Carillo MR, Cacciola NA, Shidlovskii YV, Peluso G, Digilio FA	Molecules (Basel, Switzerland)	2022
Understanding the Role of Histone Deacetylase and their Inhibitors in Neurodegenerative Disorders: Current Targets and Future Perspective Kumar V, Kundu S, Singh A, Singh S	Current neuropharmacology	2022
Rosiglitazone Ameliorates Cardiac and Skeletal Muscle Dysfunction by Correction of Energetics in Huntington's Disease. Tomczyk M, Braczko A, Mierzejewska P, Podlacha M, Krol O, Jablonska P, Jedrzejewska A, Pierzynowska K, Wegrzyn G, Slominska EM, Smolenski RT	Cells	2022
Divergent Cellular Energetics, Glutamate Metabolism, and Mitochondrial Function Between Human and Mouse Cerebral Cortex. Westi EW, Jakobsen E, Voss CM, Bak LK, Pinborg LH, Aldana BI, Andersen JV	Molecular Neurobiology	2022
Therapeutic potential of melatonin and its derivatives in aging and neurodegenerative diseases. Verma AK, Singh S, Rizvi SI	Biogerontology	2022
Mitochondrial Medicine: Genetic Underpinnings and Disease Modeling Using Induced Pluripotent Stem Cell Technology PK Kargaran, D Mosqueira, T Kozicz	Frontiers in Cardiovascular Medicine	2021
Oligodendroglial Energy Metabolism and (re)Myelination V Tepavčević	Life Sciences	2021
Propensity for somatic expansion increases over the course of life in Huntington disease R Kacher, FX Lejeune, S Noël, C Cazeneuve, A Brice, S Humbert, A Durr	eLife	2021
Mitochondrial Abnormalities and Synaptic Damage in Huntington’s Disease: a Focus on Defective Mitophagy and Mitochondria-Targeted Therapeutics N Sawant, H Morton, S Kshirsagar, AP Reddy, PH Reddy	Molecular Neurobiology	2021
Metabolism in Huntington’s disease: a major contributor to pathology A Singh, N Agrawal	Metabolic Brain Disease	2021
Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease D Hu, X Sun, A Magpusao, Y Fedorov, M Thompson, B Wang, K Lundberg, DJ Adams, X Qi	Nature Communications	2021
Zebrafish an experimental model of Huntington’s disease: molecular aspects, therapeutic targets and current challenges V Kumar, C Singh, A Singh	Molecular Biology Reports	2021
Targeting N17 domain as a potential therapeutic target for the treatment of Huntington disease: An opinion Vishal Kumar, Arti Singh	EXCLI J	2021
New label-free methods for protein relative quantification applied to the investigation of an animal model of Huntington Disease F Cozzolino, A Landolfi, I Iacobucci, V Monaco, M Caterino, S Celentano, C Zuccato, E Cattaneo, M Monti, DR Borchelt	PloS one	2020
Cellular Mechanisms of Melatonin: Insight from Neurodegenerative Diseases D Chen, T Zhang, TH Lee	Biomolecules	2020
Impaired response of cerebral oxygen metabolism to visual stimulation in Huntington’s disease P Klinkmueller, M Kronenbuerger, X Miao, J Bang, KE Ultz, A Paez, X Zhang, W Duan, RL Margolis, PC van Zijl, CA Ross, J Hua	Journal of Cerebral Blood Flow & Metabolism	2020
Developmental Trajectory of Height, Weight, and BMI in Children and Adolescents at Risk for Huntington’s Disease: Effect of mHTT on Growth A Tereshchenko, E van der Plas, KD Mathews, E Epping, AL Conrad, DR Langbehn, P Nopoulos	Journal of Huntington's disease	2020
Neuronal Sigma-1 Receptors: Signaling Functions and Protective Roles in Neurodegenerative Diseases DA Ryskamp, S Korban, V Zhemkov, N Kraskovskaya, I Bezprozvanny	Frontiers in neuroscience	2019
Enhanced cerebral branched-chain amino acid metabolism in R6/2 mouse model of Huntington’s disease JV Andersen, NH Skotte, BI Aldana, A Nørremølle, HS Waagepetersen	Cellular and Molecular Life Sciences	2019
Mitochondrial Dysfunction in Huntington’s Disease; Interplay Between HSF1, p53 and PGC-1α Transcription Factors TA Intihar, EA Martinez, R Gomez-	Frontiers in cellular neuroscience	2019
The long non-coding RNA Cerox1 is a post transcriptional regulator of mitochondrial complex I catalytic activity TM Sirey, K Roberts, W Haerty, O Bedoya-Reina, S Rogatti-Granados, JY Tan, N Li, LC Heather, RN Carter, S Cooper, AJ Finch, J Wills, NM Morton, AC Marques, CP Ponting	eLife	2019
Metabolic Reprogramming in Astrocytes Distinguishes Region-Specific Neuronal Susceptibility in Huntington Mice AA Polyzos, DY Lee, R Datta, M Hauser, H Budworth, A Holt, S Mihalik, P Goldschmidt, K Frankel, K Trego, MJ Bennett, J Vockley, K Xu, E Gratton, CT McMurray	Cell Metabolism	2019
Twist1 Plays an Anti-apoptotic Role in Mutant Huntingtin Expression Striatal Progenitor Cells WP Jen, HM Chen, YS Lin, Y Chern, YC Lee	Molecular Neurobiology	2019
Bioenergetic deficits in Huntington’s disease iPSC-derived neural cells and rescue with glycolytic metabolites AJ Kedaigle, E Fraenkel, RS Atwal, M Wu, JF Gusella, ME MacDonald, JA Kaye, S Finkbeiner, VB Mattis, CM Tom, C Svendsen, AR King, Y Chen, JT Stocksdale, RG Lim, M Casale, PH Wang, LM Thompson, SS Akimov, T Ratovitski, N Arbez, CA Ross	Human Molecular Genetics	2019
Manganese Acts upon Insulin/IGF Receptors to Phosphorylate AKT and Increase Glucose Uptake in Huntington’s Disease Cells MR Bryan, KD Nordham, DI Rose, MT OBrien, P Joshi, AM Foshage, FM Gonçalves, R Nitin, MA Uhouse, M Aschner, AB Bowman	Molecular Neurobiology	2019
Metformin reverses early cortical network dysfunction and behavior changes in Huntington's disease I Arnoux, M Willam, N Griesche, J Krummeich, H Watari, N Offermann, S Weber, PN Dey, C Chen, O Monteiro, S Buettner, K Meyer, D Bano, K Radyushkin, R Langston, JJ Lambert, E Wanker, A Methner, S Krauss, S Schweiger, A Stroh	eLife	2018
N6-Furfuryladenine is protective in Huntington’s disease models by signaling huntingtin phosphorylation LE Bowie, T Maiuri, M Alpaugh, M Gabriel, N Arbez, D Galleguillos, CL Hung, S Patel, J Xia, NT Hertz, CA Ross, DW Litchfield, S Sipione, R Truant	Proceedings of the National Academy of Sciences	2018
Thermodynamics in Neurodegenerative Diseases: Interplay Between Canonical WNT/Beta-Catenin Pathway–PPAR Gamma, Energy Metabolism and Circadian Rhythms A Vallée, Y Lecarpentier, R Guillevin, JN Vallée	NeuroMolecular Medicine	2018
Hypothalamic Alterations in Neurodegenerative Diseases and Their Relation to Abnormal Energy Metabolism P Vercruysse, D Vieau, D Blum, Å Petersén, L Dupuis	Frontiers in molecular neuroscience	2018
Sodium selenite protects from 3-nitropropionic acid-induced oxidative stress in cultured primary cortical neurons D Colle, DB Santos, V de Souza, MW Lopes, RB Leal, P de Souza Brocardo, M Farina	Molecular Biology Reports	2018
VCP cooperates with UBXD1 to degrade mitochondrial outer membrane protein MCL1 in model of Huntington's disease X Guo, X Qi	Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease	2017
Huntington Disease as a Neurodevelopmental Disorder and Early Signs of the Disease in Stem Cells K Wiatr, WJ Szlachcic, M Trzeciak, M Figlerowicz, M Figiel	Molecular Neurobiology	2017
Oxidative Stress in Neurodegenerative Diseases: From Molecular Mechanisms to Clinical Applications Z Liu, T Zhou, AC Ziegler, P Dimitrion, L Zuo	Oxidative medicine and cellular longevity	2017
Optimization of trans-Splicing for Huntington's Disease RNA Therapy H Rindt, CM Tom, CL Lorson, VB Mattis	Frontiers in neuroscience	2017
Histone Deacetylase Inhibitors Protect Against Pyruvate Dehydrogenase Dysfunction in Huntington's Disease L Naia, T Cunha-Oliveira, J Rodrigues, TR Rosenstock, A Oliveira, M Ribeiro, C Carmo, SI Oliveira-Sousa, AI Duarte, MR Hayden, AC Rego	The Journal of neuroscience : the official journal of the Society for Neuroscience	2017
Melatonin as a mitochondrial protector in neurodegenerative diseases. Wongprayoon P, Govitrapong P	Cellular and molecular life sciences : CMLS	2017
Oxidative Stress and Huntington’s Disease: The Good, The Bad, and The Ugly A Kumar, RR Ratan	Journal of Huntington's disease	2016
Integration of targeted metabolomics and transcriptomics identifies deregulation of phosphatidylcholine metabolism in Huntington’s disease peripheral blood samples A Mastrokolias, R Pool, E Mina, KM Hettne, E Duijn, RC van der Mast, GJ Ommen, PA Hoen, C Prehn, J Adamski, W Roon-Mom	Metabolomics	2016
The chicken or the egg: mitochondrial dysfunction as a cause or consequence of toxicity in Huntington’s disease AA Polyzos, CT McMurray	Mechanisms of Ageing and Development	2016
The P42 peptide and Peptide-based therapies for Huntington’s disease C Marelli, F Maschat	Orphanet Journal of Rare Diseases	2016
Altered lipid metabolism in Drosophila model of Huntington’s disease K Aditi, MN Shakarad, N Agrawal	Scientific Reports	2016
The phasor-FLIM fingerprints reveal shifts from OXPHOS to enhanced glycolysis in Huntington Disease S Sameni, A Syed, JL Marsh, MA Digman	Scientific Reports	2016
Cross-phenotype association tests uncover genes mediating nutrient response in Drosophila CS Nelson, JN Beck, KA Wilson, ER Pilcher, P Kapahi, RB Brem	BMC Genomics	2016
Mitochondrial Biology and Neurological Diseases Arun S, Liu L, Donmez G	Current neuropharmacology	2016
High Protein Diet and Huntington's Disease CM Chen, YS Lin, YR Wu, P Chen, FJ Tsai, CL Yang, YT Tsao, W Chang, IS Hsieh, Y Chern, BW Soong, P Gonzalez-Alegre	PloS one	2015
The role of DNA base excision repair in brain homeostasis and disease M Akbari, M Morevati, D Croteau, VA Bohr	DNA repair	2015
Succinobucol, a Lipid-Lowering Drug, Protects Against 3-Nitropropionic Acid-Induced Mitochondrial Dysfunction and Oxidative Stress in SH-SY5Y Cells via Upregulation of Glutathione Levels and Glutamate Cysteine Ligase Activity D Colle, DB Santos, JM Hartwig, M Godoi, DF Engel, AF de Bem, AL Braga, M Farina	Molecular Neurobiology	2015
Enhanced Neuronal Glucose Transporter Expression Reveals Metabolic Choice in a HD Drosophila Model MT Besson, K Alegría, P Garrido-Gerter, LF Barros, JC Liévens, KM Iijima	PloS one	2015
Scalable Production in Human Cells and Biochemical Characterization of Full-Length Normal and Mutant Huntingtin B Huang, T Lucas, C Kueppers, X Dong, M Krause, A Bepperling, J Buchner, H Voshol, A Weiss, B Gerrits, S Kochanek, S Humbert	PloS one	2015
Abnormal Weight and Body Mass Index in Children with Juvenile Huntington’s Disease A Tereshchenko, M McHugh, JK Lee, P Gonzalez-Alegre, K Crane, J Dawson, P Nopoulos	Journal of Huntington's disease	2015
Hypothalamic overexpression of mutant huntingtin causes dysregulation of brown adipose tissue R Soylu-Kucharz, N Adlesic, B Baldo, D Kirik, Å Petersén	Scientific Reports	2015
Beyond the redox imbalance: Oxidative stress contributes to an impaired GLUT3 modulation in Huntington's disease A Covarrubias-Pinto, P Moll, M Solís-Maldonado, AI Acuña, A Riveros, MP Miró, E Papic, FA Beltrán, C Cepeda, II Concha, S Brauchi, MA Castro	Free radical biology & medicine	2015
Does PGC1α/FNDC5/BDNF Elicit the Beneficial Effects of Exercise on Neurodegenerative Disorders? MJ Farshbaf, K Ghaedi, TL Megraw, J Curtiss, MS Faradonbeh, P Vaziri, MH Nasr-Esfahani	NeuroMolecular Medicine	2015
A shared mechanism of muscle wasting in cancer and Huntington’s disease M Mielcarek, M Isalan	Clinical and Translational Medicine	2015
Huntingtin protein is essential for mitochondrial metabolism, bioenergetics and structure in murine embryonic stem cells I Ismailoglu, Q Chen, M Popowski, L Yang, SS Gross, AH Brivanlou	Developmental Biology	2014
Impaired brain energy metabolism in the BACHD mouse model of Huntington’s disease: critical role of astrocyte–neuron interactions L Boussicault, AS Hérard, N Calingasan, F Petit, C Malgorn, N Merienne, C Jan, MC Gaillard, R Lerchundi, LF Barros, C Escartin, T Delzescaux, J Mariani, P Hantraye, MF Beal, E Brouillet, C Véga, G Bonvento	Journal of Cerebral Blood Flow & Metabolism	2014
Inhibition of mitochondrial protein import by mutant huntingtin H Yano, SV Baranov, OV Baranova, J Kim, Y Pan, S Yablonska, DL Carlisle, RJ Ferrante, AH Kim, RM Friedlander	Nature Neuroscience	2014
Prospects for neuroprotective therapies in prodromal Huntington's disease: Neuroprotective Therapies In Prodromal HD A Chandra, A Johri, MF Beal	Movement disorders : official journal of the Movement Disorder Society	2014
Antioxidant gene therapy against neuronal cell death J Navarro-Yepes, L Zavala-Flores, A Anandhan, F Wang, M Skotak, N Chandra, M Li, A Pappa, D Martinez-Fong, LM Razo, B Quintanilla-Vega, R Franco	Pharmacology & Therapeutics	2014
Two-point magnitude MRI for rapid mapping of brown adipose tissue and its application to the r6/2 mouse model of huntington disease KS Lindenberg, P Weydt, HP Müller, A Bornstedt, AC Ludolph, GB Landwehrmeyer, W Rottbauer, J Kassubek, V Rasche	PloS one	2014
Altered Ca2+ signaling in skeletal muscle fibers of the R6/2 mouse, a model of Huntington's disease P Braubach, M Orynbayev, Z Andronache, T Hering, GB Landwehrmeyer, KS Lindenberg, W Melzer	The Journal of General Physiology	2014
The optic nerve: A “mito-window” on mitochondrial neurodegeneration A Maresca, C Morgia, L Caporali, ML Valentino, V Carelli	Molecular and cellular neurosciences	2013
Huntington's disease: underlying molecular mechanisms and emerging concepts J Labbadia, RI Morimoto	Trends in Biochemical Sciences	2013
PGC-1α, Mitochondrial Dysfunction and Huntington's Disease A Johri, A Chandra, MF Beal	Free radical biology & medicine	2013
A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin M Damiano, E Diguet, C Malgorn, M D'Aurelio, L Galvan, F Petit, L Benhaim, M Guillermier, D Houitte, N Dufour, P Hantraye, JM Canals, J Alberch, T Delzescaux, N Deglon, MF Beal, E Brouillet	Human Molecular Genetics	2013
A Structurally Simplified Analogue of Geldanamycin Exhibits Neuroprotective Activity MM Madathil, OM Khdour, J Jaruvangsanti, SM Hecht	ACS Medicinal Chemistry Letters	2013
Energy Failure: Does It Contribute to Neurodegeneration? D Pathak, A Berthet, K Nakamura	Annals of Neurology	2013
Relationship of Mediterranean diet and caloric intake to phenoconversion in Huntington disease K Marder, Y Gu, S Eberly, CM Tanner, N Scarmeas, D Oakes, I Shoulson,	JAMA neurology	2013
Distinct pools of non-glycolytic substrates differentiate brain regions and prime region-specific responses of mitochondria DY Lee, Z Xun, V Platt, H Budworth, CA Canaria, CT McMurray	PloS one	2013
Probucol increases striatal glutathione peroxidase activity and protects against 3-nitropropionic acid-induced pro-oxidative damage in rats D Colle, DB Santos, EL Moreira, JM Hartwig, AA Santos, LT Zimmermann, MA Hort, M Farina	PloS one	2013
Targeting sirtuin-1 in Huntington's disease: rationale and current status W Duan	CNS Drugs	2013
pH as a biomarker of neurodegeneration in Huntington's disease: a translational rodent-human MRS study MM Chaumeil, J Valette, C Baligand, E Brouillet, P Hantraye, G Bloch, V Gaura, A Rialland, P Krystkowiak, C Verny, P Damier, P Remy, AC Bachoud-Levi, P Carlier, V Lebon	Journal of Cerebral Blood Flow & Metabolism	2012
The search for sensitive biomarkers in presymptomatic Huntington disease PG Henry, F Mochel	Journal of Cerebral Blood Flow & Metabolism	2012
Genetic Correction of Huntington's Disease Phenotypes in Induced Pluripotent Stem Cells MC An, N Zhang, G Scott, D Montoro, T Wittkop, S Mooney, S Melov, LM Ellerby	Cell Stem Cell	2012
Transducer of regulated CREB-binding proteins (TORCs) transcription and function is impaired in Huntington's disease RK Chaturvedi, T Hennessey, A Johri, SK Tiwari, D Mishra, S Agarwal, YS Kim, MF Beal	Human Molecular Genetics	2012
The expanding world of stem cell modeling of Huntington's disease: creating tools with a promising future JM Ward, AR la Spada	Genome Medicine	2012
Measures of growth in children at risk for Huntington disease JK Lee, K Mathews, B Schlaggar, J Perlmutter, JS Paulsen, E Epping, L Burmeister, P Nopoulos	Neurology	2012
trans-(-)-ε-Viniferin increases mitochondrial sirtuin 3 (SIRT3), activates AMP-activated protein kinase (AMPK), and protects cells in models of Huntington Disease J Fu, J Jin, RH Cichewicz, SA Hageman, TK Ellis, L Xiang, Q Peng, M Jiang, N Arbez, K Hotaling, CA Ross, W Duan	The Journal of biological chemistry	2012
R6/2 Huntington's disease mice develop early and progressive abnormal brain metabolism and seizures E Cepeda-Prado, S Popp, U Khan, D Stefanov, J Rodríguez, LB Menalled, D Dow-Edwards, SA Small, H Moreno	The Journal of neuroscience : the official journal of the Society for Neuroscience	2012
Energizing the Mind and Body P Kievit, JQ Purnell	Diabetes	2012
IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington’s disease Marianna Sadagurski, Zhiyong Cheng, Aldo Rozzo, Isabella Palazzolo, Gregory Kelley, Xiaocheng Dong, Dmitri Krainc, Morris White	Journal of Clinical Investigation	2011
The importance of integrating basic and clinical research towards the development of new therapies for Huntington’s disease Ignacio Munoz-Sanjuan, gillain bates	Journal of Clinical Investigation	2011
Huntington’s Disease and Striatal Signaling E Roze, E Cahill, E Martin, C Bonnet, P Vanhoutte, S Betuing, J Caboche	Frontiers in neuroanatomy	2011
Early alterations of brain cellular energy homeostasis in Huntington disease models F Mochel, B Durant, X Meng, J O'Callaghan, H Yu, E Brouillet, VC Wheeler, S Humbert, R Schiffmann, A Durr	The Journal of biological chemistry	2011
Should metabolic diseases be systematically screened in nonsyndromic autism spectrum disorders? M Schiff, JF Benoist, S Aïssaoui, O Boespflug-Tanguy, O Boepsflug-Tanguy, MC Mouren, HO de Baulny, R Delorme	PloS one	2011
Pulmonary surfactant: an immunological perspective ZC Chroneos, Z Sever-Chroneos, VL Shepherd	Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology	2009

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