Results 131 to 140 of about 9,196 (170)

An integrative neuropharmacological review of Huntington's disease challenges and the role of novel formulations in addressing pharmacological‒pharmaceutical limitations. [PDF]

Front Pharmacol
Senousy MA, Eid AH, Zaki MB, A Abd-Elmawla M, Ghaiad HR, El-Shiekh RA, Ahmed S, Khalifa HO.   +7 more
europepmc   +1 more source

Translational Relevance of SCA1 Models for the Development of Therapies for Spinocerebellar Ataxia Type 1. [PDF]

Biomedicines
Plotnikova E, Ageeva T, Sufianov A, Sufianova G, Rizvanov A, Mukhamedshina Y.   +5 more
europepmc   +1 more source

Restoring early postnatal synaptic dysregulation rescues motor neuron degeneration in a mouse model of Spinal and Bulbar Muscular Atrophy. [PDF]

Nat Commun
Hirunagi T, Sahashi K, Iida M, Onodera K, Yokoi S, Ogura Y, Tohnai G, Sakakibara K, Maeda K, Bennett CF, Okada Y, Katsuno M.   +11 more
europepmc   +1 more source

Visualization of autophagic structures near solid polyQ aggregates reveals how they undermine autophagy. [PDF]

Autophagy
Popelka H, Klionsky DJ.
europepmc   +1 more source

An acyclic nucleic acid-modified siRNA targeting CAG expansions for polyglutamine disease treatment. [PDF]

Mol Ther Nucleic Acids
Maeda K, Hirunagi T, Sahashi K, Kamiya Y, Iida M, Sakakibara K, Onodera K, Ohyama M, Okada Y, Okano H, Asanuma H, Katsuno M.   +11 more
europepmc   +1 more source

Polyglutamine (polyQ) disorders [PDF]

Nucleus, 2012
Polyglutamine (PolyQ)-related diseases are dominant late-onset genetic disorders that are manifested by progressive neurodegeneration, leading to behavioral and physical impairments. An increased body of evidence suggests that chromatin structure and epigenetic regulation are involved in disease pathology.
Eran Meshorer
exaly   +3 more sources

Polyglutamine Disorders [PDF]

Advances in Experimental Medicine and Biology, 2018
exaly   +2 more sources

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Neurotoxicity Pathways in Drosophila Models of the Polyglutamine Disorders

Current Topics in Developmental Biology, 2017
Although polyglutamine expansion diseases are the most common genetically inherited neurodegenerative disorders, the key pathogenic mechanisms that lead to neuronal cell death are unclear. The expansion of a polyglutamine tract in specific proteins is the defining molecular insult, leading to cell-type and region-specific neuronal death.
J Troy Littleton
exaly   +3 more sources

Role of proteolysis in polyglutamine disorders

Journal of Neuroscience Research, 2003
AbstractTo date, nine polyglutamine disorders have been characterised, including Huntington's disease (HD), spinobulbar muscular atrophy (SBMA), dentatorubral‐pallidoluysian atrophy (DRPLA), and spinocerebellar ataxias 1, 2, 3, 6, 7 and 17 (SCAs). Although knockout and transgenic mouse experiments suggest that a toxic gain of function is central to ...
Elsdon Storey
exaly   +3 more sources

Epigenetic profiles in polyglutamine disorders

Epigenomics, 2017
The dominant polyglutamine (polyQ) disorders are a group of progressive and incurable neurodegenerative disorders, which are caused by unstable expanded CAG trinucleotide repeats in the coding regions of their respective causative genes. The most prevalent polyQ disorders worldwide are Huntington's disease and spinocerebellar ataxia type 3.
Hongmei, Liu, Tie-Shan, Tang, Caixia, Guo   +2 more
openaire   +2 more sources