Results 91 to 100 of about 37,064 (267)

Neuroprotective effects of ursodeoxycholic acid in Parkinson's disease and Alzheimer's disease

Neuroprotection, EarlyView.
In Parkinson's disease (PD) and Alzheimer's disease (AD), various shared pathological mechanisms exist. UDCA has been shown to exert neuroprotective effects in preclinical and clinical studies. Such effects include the mitigation of neuroinflammation as well as rescue effects on mitochondrial dysfunction, oxidative stress, impaired autophagy, and ...
Ashley En Yi Chong, Andrew Octavian Sasmita, Rhun Yian Koh, Anna Pick Kiong Ling   +3 more
wiley   +1 more source

Proteasome activator enhances survival of Huntington's disease neuronal model cells. [PDF]

PLoS ONE, 2007
In patients with Huntington's disease (HD), the proteolytic activity of the ubiquitin proteasome system (UPS) is reduced in the brain and other tissues. The pathological hallmark of HD is the intraneuronal nuclear protein aggregates of mutant huntingtin.
Hyemyung Seo, Kai-Christian Sonntag, Woori Kim, Elena Cattaneo, Ole Isacson   +4 more
doaj   +1 more source

Rapamycin reduces neuronal mutant huntingtin aggregation and ameliorates locomotor performance in Drosophila

Frontiers in Aging Neuroscience, 2023
Huntington’s disease (HD) is a neurodegenerative disease characterized by movement and cognitive dysfunction. HD is caused by a CAG expansion in exon 1 of the HTT gene that leads to a polyglutamine (PQ) repeat in the huntingtin protein, which aggregates ...
Jonathan R. Roth, Jonathan R. Roth, Ruan Carlos Macedo de Moraes, Brittney P. Xu, Savannah R. Crawley, Malghalara A. Khan, Girish C. Melkani   +6 more
doaj   +1 more source

The Neuroprotective Effect of a Waste Byproduct Obtained From Pomegranate (Punica granatum)

Phytotherapy Research, EarlyView.
The Effect of a Waste Byproduct Obtained From Pomegranate on Neurodegeneration. ABSTRACT Pomegranate is an exceptional fruit that can have several beneficial effects on human health. The peel of pomegranate, a waste product, should be recovered as it still contains valuable constituents, including phenolic compounds, minerals and fibre. The recovery of
Jessica Maiuolo, Valeria Mazza, Rosamaria Caminiti, Francesca Oppedisano, Saverio Nucera, Salvatore Ragusa, Sara Ilari, Lucia Carmela Passacatini, Luigi Tucci, Giuseppe Trunfio, Vincenzo Mollace, Carolina Muscoli   +11 more
wiley   +1 more source

Fibrillogenesis of Huntingtin and Other Glutamine Containing Proteins [PDF]

, 2012
This chapter focuses on the aggregation of glutamine containing peptides and proteins with an emphasis on huntingtin protein, whose aggregation leads to the development of Huntington's disease. The kinetics that leads to the formation of amyloids, the structure of aggregates of various types and the morphological mechanical properties of amyloid ...
Yuri L, Lyubchenko, Alexey V, Krasnoslobodtsev, Sorin, Luca   +2 more
openaire   +2 more sources

Mutant huntingtin gene-dose impacts on aggregate deposition, DARPP32 expression and neuroinflammation in HdhQ150 mice. [PDF]

PLoS ONE, 2013
Huntington's disease (HD) is an autosomal dominant, progressive and fatal neurological disorder caused by an expansion of CAG repeats in exon-1 of the huntingtin gene.
Douglas Young, Franziska Mayer, Nella Vidotto, Tatjana Schweizer, Ramon Berth, Dorothee Abramowski, Derya R Shimshek, P Herman van der Putten, Peter Schmid   +8 more
doaj   +1 more source

Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice

Brain Pathology, EarlyView.
We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga, Eva Fernández‐Muñoz, Jorge M. Charco, Enric Vidal, Diego Herrero‐Martínez, Josu Galarza‐Ahumada, Cristina Sampedro‐Torres‐Quevedo, Samanta Giler, Mariví Geijo, Gloria González‐Aseguinolaza, Hasier Eraña, Joaquín Castilla   +11 more
wiley   +1 more source

A Systems Biology Approach towards Deciphering the Unfolded Protein Response in Huntington's Disease [PDF]

, 2012
Although the disease causing gene huntingtin has been known for some time, the exact cause of neuronal cell death during _Huntington's disease_ (HD) remains unknown.
Kameshwar R. Ayasolla, Matthias E. Futschik, Ravi K. R. Kalathur   +2 more
core   +2 more sources

The DNA/RNA autophagy protein SIDT2 as a novel neuropathological hallmark in Huntington disease

Brain Pathology, EarlyView.
SIDT2‐immunoreactive inclusions are observed in the striatum, cerebral cortex, and hypothalamus in HD cases with different Vonsattel grades, and the frequency of SIDT2‐immunoreactive inclusions is associated with longer CAG repeats in the huntingtin gene.
Sanaz Gabery, Sofia Bergh, Chrisovalantou Huridou, Rachel Y. Cheong, Barbara Baldo, Paul Günther Scheunemann, Marie‐Louisa Schoebel, Linda Holmquist Mengelbier, Elisabet Englund, Catriona McLean, Carsten Saft, Deniz Kirik, Maria Björkqvist, Glenda Halliday, Elisabeth Petrasch‐Parwez, Huu Phuc Nguyen, Jonasz Jeremiasz Weber, Åsa Petersén   +17 more
wiley   +1 more source

Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. [PDF]

, 2015
Quantification of disease-associated proteins in the cerebrospinal fluid (CSF) has been critical for the study and treatment of several neurodegenerative disorders; however, mutant huntingtin protein (mHTT), the cause of Huntington's disease (HD), is at ...
Andreas Weiss, Benjamini, Blair R. Leavitt, Douglas Langbehn, Douglas Macdonald, Edward J. Wild, Henrik Zetterberg, James R.C. Miller, Jeromin, Ko, Lewczuk, Nicola Robertson, Rainer Kuhn, Roberto Boggio, Rosengren, Salman Haider, Sarah J. Tabrizi   +16 more
core   +1 more source