The cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington’s disease
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Carlos A. Matos | J. Caboche | S. Carmo-Silva | C. Nóbrega | Raquel L. Sequeira | Sandro Alves | N. Cartier | S. Betuing | Adriana Marcelo | Rafael G. Costa | Ricardo Nunes | André Conceição | Rebekah Koppenol | Clévio Nóbrega
[1] J. Caboche,et al. CYP46A1 gene therapy deciphers the role of brain cholesterol metabolism in Huntington's disease. , 2019, Brain : a journal of neurology.
[2] Carlos A. Matos,et al. Restoring brain cholesterol turnover improves autophagy and has therapeutic potential in mouse models of spinocerebellar ataxia , 2019, Acta Neuropathologica.
[3] S. Tabrizi,et al. Huntingtin Lowering Strategies for Disease Modification in Huntington’s Disease , 2019, Neuron.
[4] Carlos A. Matos,et al. Machado–Joseph disease/spinocerebellar ataxia type 3: lessons from disease pathogenesis and clues into therapy , 2018, Journal of neurochemistry.
[5] Carlos A. Matos,et al. Cordycepin activates autophagy through AMPK phosphorylation to reduce abnormalities in Machado‐Joseph disease models , 2018, Human molecular genetics.
[6] A. Brice,et al. Deregulation of autophagy in postmortem brains of Machado‐Joseph disease patients , 2018, Neuropathology : official journal of the Japanese Society of Neuropathology.
[7] Lidia Gaffke,et al. Correction of Huntington’s Disease Phenotype by Genistein-Induced Autophagy in the Cellular Model , 2018, NeuroMolecular Medicine.
[8] P. McColgan,et al. Huntington's disease: a clinical review , 2018, European journal of neurology.
[9] I. Bièche,et al. Neuronal Cholesterol Accumulation Induced by Cyp46a1 Down-Regulation in Mouse Hippocampus Disrupts Brain Lipid Homeostasis , 2017, Front. Mol. Neurosci..
[10] E. Hol,et al. Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell‐type‐specific , 2016, Glia.
[11] C. Pagès,et al. CYP46A1, the rate-limiting enzyme for cholesterol degradation, is neuroprotective in Huntington’s disease , 2016, Brain : a journal of neurology.
[12] A. Vercelli,et al. Cholesterol‐loaded nanoparticles ameliorate synaptic and cognitive function in Huntington's disease mice , 2015, EMBO molecular medicine.
[13] H. Hirai,et al. Re-establishing ataxin-2 downregulates translation of mutant ataxin-3 and alleviates Machado-Joseph disease. , 2015, Brain : a journal of neurology.
[14] R. Miles,et al. CYP46A1 inhibition, brain cholesterol accumulation and neurodegeneration pave the way for Alzheimer's disease. , 2015, Brain : a journal of neurology.
[15] D. Ehrnhoefer,et al. Autophagy in Huntington disease and huntingtin in autophagy , 2015, Trends in Neurosciences.
[16] F. Pfrieger,et al. Cholesterol in brain disease: sometimes determinant and frequently implicated , 2014, EMBO reports.
[17] V. Fung,et al. An update on Huntington's disease: from the gene to the clinic. , 2014, Current opinion in neurology.
[18] M. Priault,et al. The autophagy/lysosome pathway is impaired in SCA7 patients and SCA7 knock-in mice , 2014, Acta Neuropathologica.
[19] Jane S. Paulsen,et al. Huntington disease: natural history, biomarkers and prospects for therapeutics , 2014, Nature Reviews Neurology.
[20] N. Déglon,et al. Overexpression of Mutant Ataxin-3 in Mouse Cerebellum Induces Ataxia and Cerebellar Neuropathology , 2013, The Cerebellum.
[21] H. Hirai,et al. Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado-Joseph disease. , 2013, Brain : a journal of neurology.
[22] J. Vance. Dysregulation of cholesterol balance in the brain: contribution to neurodegenerative diseases , 2012, Disease Models & Mechanisms.
[23] J. Son,et al. Neuronal autophagy and neurodegenerative diseases , 2012, Experimental & Molecular Medicine.
[24] P. V. Van Veldhoven,et al. Regulation of tyrosine kinase B activity by the Cyp46/cholesterol loss pathway in mature hippocampal neurons: relevance for neuronal survival under stress and in aging , 2011, Journal of neurochemistry.
[25] R. Roos,et al. Huntington's disease: a clinical review , 2010, Orphanet journal of rare diseases.
[26] M. MacDonald,et al. Cholesterol Defect Is Marked across Multiple Rodent Models of Huntington's Disease and Is Manifest in Astrocytes , 2010, The Journal of Neuroscience.
[27] D. Russell,et al. Cholesterol 24-hydroxylase: an enzyme of cholesterol turnover in the brain. , 2009, Annual review of biochemistry.
[28] A. Smit,et al. SREBPs: SREBP function in glia–neuron interactions , 2009, The FEBS journal.
[29] A. Kenworthy,et al. Lipid rafts, cholesterol, and the brain , 2008, Neuropharmacology.
[30] D. Rubinsztein,et al. Novel targets for Huntington's disease in an mTOR-independent autophagy pathway. , 2008, Nature chemical biology.
[31] S. Andersson,et al. Neuronal expression and subcellular localization of cholesterol 24‐hydroxylase in the mouse brain , 2008, The Journal of comparative neurology.
[32] D. Rubinsztein,et al. A rational mechanism for combination treatment of Huntington's disease using lithium and rapamycin. , 2008, Human molecular genetics.
[33] C. Mariotti,et al. Progressive dysfunction of the cholesterol biosynthesis pathway in the R6/2 mouse model of Huntington’s disease , 2007, Neurobiology of Disease.
[34] M. Hayden,et al. Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation. , 2007, Human molecular genetics.
[35] T. Noda,et al. Dissection of the Autophagosome Maturation Process by a Novel Reporter Protein, Tandem Fluorescent-Tagged LC3 , 2007, Autophagy.
[36] I. Björkhem,et al. Crossing the barrier: oxysterols as cholesterol transporters and metabolic modulators in the brain , 2006, Journal of internal medicine.
[37] Anne E Carpenter,et al. CellProfiler: image analysis software for identifying and quantifying cell phenotypes , 2006, Genome Biology.
[38] J. Olson,et al. Dysfunction of the Cholesterol Biosynthetic Pathway in Huntington's Disease , 2005, The Journal of Neuroscience.
[39] Steve Meaney,et al. Brain Cholesterol: Long Secret Life Behind a Barrier , 2004, Arteriosclerosis, thrombosis, and vascular biology.
[40] Wilhelm Gaus,et al. Evidence for more widespread cerebral pathology in early HD: An MRI-based morphometric analysis , 2004, Neurology.
[41] N Makris,et al. Evidence for more widespread cerebral pathology in early HD , 2003, Neurology.
[42] J. Dietschy,et al. Control of Cholesterol Turnover in the Mouse* , 2002, The Journal of Biological Chemistry.
[43] D. Rubinsztein,et al. A molecular investigation of true dominance in Huntington’s disease , 1999, Journal of medical genetics.
[44] J M Guileyardo,et al. cDNA cloning of cholesterol 24-hydroxylase, a mediator of cholesterol homeostasis in the brain. , 1999, Proceedings of the National Academy of Sciences of the United States of America.
[45] J. Wahren,et al. Cholesterol homeostasis in human brain: turnover of 24S-hydroxycholesterol and evidence for a cerebral origin of most of this oxysterol in the circulation. , 1998, Journal of lipid research.
[46] A E Rosser,et al. Evidence for specific cognitive deficits in preclinical Huntington's disease. , 1998, Brain : a journal of neurology.
[47] R. Ferrante,et al. Neuropathological Classification of Huntington's Disease , 1985, Journal of neuropathology and experimental neurology.
[48] Pierre Bougnères,et al. Adeno-associated virus gene therapy with cholesterol 24-hydroxylase reduces the amyloid pathology before or after the onset of amyloid plaques in mouse models of Alzheimer's disease. , 2010, Molecular therapy : the journal of the American Society of Gene Therapy.
[49] V. Hachinski,et al. Evidence for more widespread cerebral pathology in early HD: An MRI-based morphometric analysis. Authors' reply , 2004 .
[50] Hilde van der Togt,et al. Publisher's Note , 2003, J. Netw. Comput. Appl..
[51] D. Lütjohann,et al. Evidence that the major oxysterols in human circulation originate from distinct pools of cholesterol: a stable isotope study. , 2001, Journal of lipid research.
[52] H. Zoghbi,et al. Glutamine repeats and neurodegeneration. , 2000, Annual review of neuroscience.