Results 101 to 110 of about 5,553,804 (280)
Molecular Neurodegeneration, 2010 Background Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a CAG repeat expansion within the huntingtin gene. Mutant huntingtin protein misfolds and accumulates within neurons where it mediates its toxic effects. Promoting
Frentzel Stefan +11 more
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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 +4 more
doaj +1 more source
Brain Pathology, EarlyView.Sphingolipids are vital components of cell membranes. Metabolic disruptions of sphingolipids, including ceramide and sphingosine‐1‐phosphate, are linked to neurological disorders. This article summarizes the classification, structure, and metabolic processes of sphingolipids, and the physiological and pathological effects of sphingolipid metabolism and
Tian Li +7 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 +2 more
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An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity
Cell Death and Disease, 2012 Huntington’s disease (HD) is a devastating neurodegenerative disorder whose main hallmark is brain atrophy. However, several peripheral organs are considerably affected and their symptoms may, in fact, manifest before those resulting from brain pathology.
G. Cisbani, F. Cicchetti
semanticscholar +1 more source
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 +8 more
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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 +6 more
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Beyond synapses: cytoplasmic connections in brain function and evolution
Biological Reviews, EarlyView.ABSTRACT Following Ramón y Cajal's groundbreaking contributions to the identification of synapses, research in neuroscience predominantly focused on their pivotal role in neural communication (the neuron doctrine), overlooking an intriguing possibility suggested by Golgi of non‐synaptic interactions among neural cells.
Malalaniaina Rakotobe, Chiara Zurzolo
wiley +1 more source
Suppression of polyglutamine toxicity by a Drosophila homolog of myeloid leukemia factor 1 [PDF]
, 2002 The toxicity of an abnormally long polyglutamine [poly(Q)] tract within specific proteins is the molecular lesion shared by Huntington's disease (HD) and several other hereditary neurodegenerative disorders.
Benzer, Seymour, Kazemi-Esfarjani, Parsa
core +1 more source
Mechanisms of copper ion mediated Huntington's disease progression. [PDF]
PLoS ONE, 2007 Huntington's disease (HD) is caused by a dominant polyglutamine expansion within the N-terminus of huntingtin protein and results in oxidative stress, energetic insufficiency and striatal degeneration.
Jonathan H Fox +9 more
doaj +1 more source