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Huntingtin-Associated Proteins
2001Huntington’s disease (HD), with its writhing dancelike movements (chorea) and cardinal loss of neurons in the striatum (1), is the result of an unstable expanded CAG trinucleotide repeat that lengthens a variable glutamine tract in a novel protein called huntingtin (HD) (2).
Marcy E. MacDonald +2 more
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ACS Chemical Neuroscience, 2022
Huntington's disease is a progressive and lethal neurodegenerative disease caused by an increased CAG repeat mutation in exon 1 of the huntingtin gene (mutant huntingtin). Current drug treatments provide only limited symptomatic relief without impacting disease progression.
Khushboo Kapadia +9 more
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Huntington's disease is a progressive and lethal neurodegenerative disease caused by an increased CAG repeat mutation in exon 1 of the huntingtin gene (mutant huntingtin). Current drug treatments provide only limited symptomatic relief without impacting disease progression.
Khushboo Kapadia +9 more
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Brain Research Bulletin, 2001
We produced eight anti-huntingtin (Htt) monoclonal antibodies (mAbs), several of which have novel binding patterns. Peptide array epitope mapping shows that mAbs MW1-6 specifically bind the polyQ domain of Htt exon 1. On Western blots of extracts from mutant Htt knock-in mouse brain and Huntington's disease lymphoblastoma cell lines, MW1-5 all strongly
Ko, Jan, Ou, Susan, Patterson, Paul H.
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We produced eight anti-huntingtin (Htt) monoclonal antibodies (mAbs), several of which have novel binding patterns. Peptide array epitope mapping shows that mAbs MW1-6 specifically bind the polyQ domain of Htt exon 1. On Western blots of extracts from mutant Htt knock-in mouse brain and Huntington's disease lymphoblastoma cell lines, MW1-5 all strongly
Ko, Jan, Ou, Susan, Patterson, Paul H.
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Effect of Pesticides on the Aggregation of Mutant Huntingtin Protein
Molecular Neurobiology, 2012The classical reports on neurodegeneration concentrate on studying disruption of signalling cascades. Although it is now well recognized that misfolding and aggregation of specific proteins are associated with a majority of these diseases, their role in aggravating the symptoms is not so well understood. Huntington's disease (HD) is a neurodegenerative
Ruhi S, Deshmukh +2 more
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Huntingtin–protein interactions and the pathogenesis of Huntington's disease
Trends in Genetics, 2004At least nine inherited neurodegenerative diseases share a polyglutamine expansion in their respective disease proteins. These diseases show distinct neuropathological changes, suggesting that protein environment and protein-protein interactions play an important role in the specific neuropathology.
Shi-Hua, Li, Xiao-Jiang, Li
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Aggregate-centered redistribution of proteins by mutant huntingtin
Biochemical and Biophysical Research Communications, 2007Huntingtin is a widely expressed 350-kDa cytosolic multidomain of unknown function. Aberrant expansion of the polyglutamine tract located in the N-terminal region of huntingtin results in Huntington's disease. The presence of insoluble huntingtin inclusions in the brains of patients is one of the hallmarks of Huntington's disease.
Leigh Anne, Swayne, Janice E A, Braun
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Huntingtin interacting protein HYPK is intrinsically unstructured
Proteins: Structure, Function, and Bioinformatics, 2007AbstractTo characterize HYPK, originally identified as a novel huntingtin (Htt) interacting partner by yeast two hybrid assay, we used various biophysical and biochemical techniques. The molecular weight of the protein, determined by gel electrophoresis, was found to be about 1.3‐folds (∼22 kDa) higher than that obtained from mass spectrometric ...
Swasti, Raychaudhuri +5 more
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Huntingtin is associated with cytomatrix proteins at the presynaptic terminal
Molecular and Cellular Neuroscience, 2014Huntington's disease (HD) is a single gene disorder produced by expansion of the gene encoding huntingtin (htt), a large protein with features of a multi-functional scaffold. Expansion of htt's polyglutamine domain induces novel, toxic interactions and likely also disrupts normal htt function.
Jia, Yao +2 more
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Apoptotic Regulation by the Huntingtin Protein
2001Title page and contents Chapter I - Introduction 1 A Matter of Death 1 1.1 Necrosis 1 1.1.1 Morphological Features of Necrosis 2 1.2 Apoptosis 2 1.2.1 Morphological Features of Apoptosis 3 1.3 Apoptosis and Necrosis: A Continuum? 4 2 The Molecular Components of Apoptosis 2.1 Death Receptors 5 2.2 Bcl-2 Family 6 2.3 Apaf-1 Family 7 2.4 Caspases 7 2.5 ...
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[Protein-Protein Interactions of Huntingtin in the Hippocampus].
Molekuliarnaia biologiia, 2018Huntingtin (HTT) occurs in the neuronal cytoplasm and can interact with structural elements of synapses. Huntington's disease (HD) results from pathological expansion of a polyglutamine stretch in the HTT molecule, being probably associated with aberrant protein-protein interactions.
A L, Proskura +3 more
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