Results 31 to 40 of about 21,717 (167)

Transient Interdomain Interactions Modulate the Monomeric Structural Ensemble and Self‐Assembly of Huntingtin Exon 1

Advanced Science, EarlyView.
Polyglutamine (polyQ) tract expansion (≥ 36 amino acids) within the N‐terminal region of the Huntingtin protein (Httex1) causes Huntington's disease (HD), for which the underlying causes are not well‐understood. The authors performed computer simulations to understand the cause of HD at the molecular level.
Priyesh Mohanty, Tien Minh Phan, Jeetain Mittal   +2 more
wiley   +1 more source

Tissue Transglutaminase Selectively Modifies Proteins Associated with Truncated Mutant Huntingtin in Intact Cells

Neurobiology of Disease, 2001
The cause of Huntington's disease (HD) is a pathological expansion of the polyglutamine domain within the N-terminal region of huntingtin. Neuronal intranuclear inclusions and cytoplasmic aggregates composed of the mutant huntingtin within certain ...
Wanjoo Chun, Mathieu Lesort, Janusz Tucholski, Peter W. Faber, Marcy E. MacDonald, Christopher A. Ross, Gail V.W. Johnson   +6 more
doaj  

ER stress-induced eIF2-alpha phosphorylation underlies sensitivity of striatal neurons to pathogenic huntingtin.

PLoS ONE, 2014
A hallmark of Huntington's disease is the pronounced sensitivity of striatal neurons to polyglutamine-expanded huntingtin expression. Here we show that cultured striatal cells and murine brain striatum have remarkably low levels of phosphorylation of ...
Julia Leitman, Boaz Barak, Ron Benyair, Marina Shenkman, Uri Ashery, F Ulrich Hartl, Gerardo Z Lederkremer   +6 more
doaj   +1 more source

PolyQ-Expansion Causes Mitochondria Fragmentation Independent of Huntingtin and Is Distinct from Traumatic Brain Injury (TBI)/Mechanical Stress-Mediated Fragmentation Which Results from Cell Death

Cells, 2023
Mitochondrial dysfunction has been reported in many Huntington’s disease (HD) models; however, it is unclear how these defects occur. Here, we test the hypothesis that excess pathogenic huntingtin (HTT) impairs mitochondrial homeostasis, using Drosophila
Kelsey Swinter, Dania Salah, Rasika Rathnayake, Shermali Gunawardena   +3 more
doaj   +1 more source

Solving the Amyloid Paradox: Unveiling the Complex Pathogenicity of Amyloid Fibrils

Aggregate, EarlyView.
This review addresses the gap between strong evidence for the involvement of amyloid fibrils in neurodegeneration and the failure of anti‐amyloid therapies, a phenomenon herein termed the “amyloid paradox.” To address this paradox, we provide a comprehensive summary of the current understanding of fibrils' pathogenic properties and mechanisms ...
Maksim I. Sulatsky, Olesya V. Stepanenko, Olga V. Stepanenko, Anna I. Sulatskaya   +3 more
wiley   +1 more source

Exploring the Neuroprotective Role of Selenium: Implications and Perspectives for Central Nervous System Disorders

Exploration, EarlyView.
Selenium (Se) is a crucial element in selenoproteins, key biomolecules for physiological function in vivo. Central nervous system can express all 25 kinds of selenoproteins, which protect neurons by reducing oxidative stress and inflammatory response. Neuroprotection is being investigated through the biological study of Se.
Guanning Huang, Ying Liu, Xiadong Zhu, Lizhen He, Tianfeng Chen   +4 more
wiley   +1 more source

Genetic and pharmacological inhibition of calcineurin corrects the BDNF transport defect in Huntington's disease

Molecular Brain, 2009
Background Huntington's disease (HD) is an inherited neurogenerative disease caused by an abnormal expansion of glutamine repeats in the huntingtin protein.
Pineda Jose R, Pardo Raúl, Zala Diana, Yu Hua, Humbert Sandrine, Saudou Frédéric   +5 more
doaj   +1 more source

Mitochondrial dysfunction: Related diseases, influencing factors, and detection

Interdisciplinary Medicine, EarlyView.
Mitochondrial dysfunction is implicated in the pathogenesis of numerous diseases, including neurological disorders, cancers, and cardiovascular conditions, through mechanisms such as mitochondrial DNA mutations, dysregulation of mitochondrial network dynamics, and impaired mitophagy.
Zhaojin Li, Shiyu Li, Wanying Chen, Ziyu Zhang, Chun Pan, Peng Lei, Cheng Cheng, Junyou Zhu, Hanxiao Sun, Zhenning Dai   +9 more
wiley   +1 more source

Preserved Thermoregulation in Huntington's Disease: Insights from an Observational Case–Control Study

Movement Disorders Clinical Practice, EarlyView.
ABSTRACT Background Thermoregulation is critical for preventing hyperthermia during physical activity. In Huntington's disease (HD), research on possible thermoregulatory alterations has received little attention. Objectives We aimed to investigate and compare skin temperature differences before and after exercise between HD patients and healthy ...
Lucía Simón‐Vicente, Jéssica Rivadeneyra‐Posadas, María Soto‐Célix, Javier Raya‐González, Alejandro Rodríguez‐Fernández, Daniel Castillo‐Alvira, Fernando Vázquez, Esther Cubo   +7 more
wiley   +1 more source

Reduction of mutant huntingtin accumulation and toxicity by lysosomal cathepsins D and B in neurons

Molecular Neurodegeneration, 2011
Background Huntington's disease is caused by aggregation of mutant huntingtin (mHtt) protein containing more than a 36 polyQ repeat. Upregulation of macroautophagy was suggested as a neuroprotective strategy to degrade mutant huntingtin.
Ouyang Xiaosen, Liang Qiuli, Schneider Lonnie, Zhang Jianhua   +3 more
doaj   +1 more source