Results 121 to 130 of about 28,868 (258)

Raft-like microdomains play a key role in mitochondrial impairment in lymphoid cells from patients with Huntington's disease

Journal of Lipid Research, 2012
Huntington's disease (HD) is a genetic neurodegenerative disease characterized by an exceedingly high number of contiguous glutamine residues in the translated protein, huntingtin (Htt).
Laura Ciarlo, Valeria Manganelli, Paola Matarrese, Tina Garofalo, Antonella Tinari, Lucrezia Gambardella, Matteo Marconi, Maria Grasso, Roberta Misasi, Maurizio Sorice, Walter Malorni   +10 more
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Loss-of-Huntingtin in Medial and Lateral Ganglionic Lineages Differentially Disrupts Regional Interneuron and Projection Neuron Subtypes and Promotes Huntington's Disease-Associated Behavioral, Cellular, and Pathological Hallmarks

Journal of Neuroscience, 2019
Emerging studies are providing compelling evidence that the pathogenesis of Huntington's disease (HD), a neurodegenerative disorder with frequent midlife onset, encompasses developmental components.
M. Mehler, Jenna R. Petronglo, Eduardo E. Arteaga-Bracho, M. Gulinello, Michael L Winchester, N. Pichamoorthy, Stephen K. Young, Christopher D. Dejesus, Hifza Ishtiaq, Solen Gokhan, A. Molero   +10 more
semanticscholar   +1 more source

Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity

Brain Sciences, 2017
Huntington’s disease (HD) is an autosomal dominant neurodegenerative disease caused by the expansion of a CAG trinucleotide repeat encoding an abnormally long polyglutamine tract (PolyQ) in the huntingtin (Htt) protein.
Ryoma Morigaki, Satoshi Goto
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Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo [PDF]

, 1997
Eberhard Scherzinger, Rudi Lurz, Mark Turmaine, Laura Mangiarini, Birgit Hollenbach, Renate Hasenbank, Gillian P. Bates, Stephen W. Davies, Hans Lehrach, Erich E. Wanker   +9 more
openalex   +1 more source

Oral administration of the cannabigerol derivative VCE-003.2 promotes subventricular zone neurogenesis and protects against mutant huntingtin-induced neurodegeneration

Translational Neurodegeneration, 2019
The administration of certain cannabinoids provides neuroprotection in models of neurodegenerative diseases by acting through various cellular and molecular mechanisms.
J. Aguareles, Juan Paraíso-Luna, Belén Palomares, R. Bajo‐Grañeras, C. Navarrete, A. Ruiz-Calvo, D. García-Rincón, E. García‐Taboada, M. Guzmán, E. Muñoz, I. Galve-Roperh   +10 more
semanticscholar   +1 more source

Molecular mechanisms of heterogeneous oligomerization of huntingtin proteins

Scientific Reports, 2019
There is still no successful strategy to treat Huntington’s disease, an inherited autosomal disorder associated with the aggregation of mutated forms of the huntingtin protein containing polyglutamine tracts with more than 36 repeats. Recent experimental
S. Bonfanti, M. C. Lionetti, M. R. Fumagalli, Venkat R. Chirasani, G. Tiana, N. Dokholyan, S. Zapperi, C. L. La Porta   +7 more
semanticscholar   +1 more source

N6-Furfuryladenine is protective in Huntington’s disease models by signaling huntingtin phosphorylation

Proceedings of the National Academy of Sciences of the United States of America, 2018
Significance We have discovered a molecule derived from DNA-damage repair that can correct the lack of phosphorylation of mutant huntingtin, the cause of Huntington’s disease (HD).
L. Bowie, Tamara Maiuri, M. Alpaugh, Michelle Gabriel, N. Arbez, D. Galleguillos, C. L. Hung, Shreya A. Patel, Jianrun Xia, Nicholas T. Hertz, C. Ross, D. Litchfield, S. Sipione, R. Truant   +13 more
semanticscholar   +1 more source

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin [published erratum appears in Hum Mol Genet 1999 May;8(5):943] [PDF]

, 1999
Gabriele Schilling
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Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies. [PDF]

, 1995
C A Gutekunst, Allan I. Levey, C J Heilman, William L. Whaley, Hong Yi, N. R. Nash, Howard D. Rees, John J. Madden, Steven M. Hersch   +8 more
openalex   +1 more source

Polyglutamine Repeat Length-Dependent Proteolysis of Huntingtin

Neurobiology of Disease, 2002
Amino-terminal fragments of huntingtin, which contain the expanded polyglutamine repeat, have been proposed to contribute to the pathology of Huntington's disease (HD).
Banghua Sun, Wei Fan, Aldona Balciunas, Jillian K. Cooper, Gal Bitan, Shirley Steavenson, Paul E. Denis, Yunjen Young, Beverly Adler, Larry Daugherty, Raffi Manoukian, Gary Elliott, Wenyan Shen, Jane Talvenheimo, David B. Teplow, Mitsuru Haniu, Raj Haldankar, Jette Wypych, Christopher A. Ross, Martin Citron, William G. Richards   +20 more
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