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Huntington Disease and the Huntingtin Protein
2012Huntington disease (HD) is a devastating neurodegenerative disease that derives from CAG repeat expansion in the huntingtin gene. The clinical syndrome consists of progressive personality changes, movement disorder, and dementia and can develop in children and adults.
Zhiqiang, Zheng, Marc I, Diamond
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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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Cellular and Molecular Life Sciences, 2022
Huntingtin-associated protein 1 (HAP1) is the first identified protein whose function is affected by its abnormal interaction with mutant huntingtin (mHTT), which causes Huntington disease. However, the expression patterns of Hap1 and Htt in the rodent brain are not correlated.
Xingxing Chen +15 more
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Huntingtin-associated protein 1 (HAP1) is the first identified protein whose function is affected by its abnormal interaction with mutant huntingtin (mHTT), which causes Huntington disease. However, the expression patterns of Hap1 and Htt in the rodent brain are not correlated.
Xingxing Chen +15 more
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Protein folding: Sticky N17 speeds huntingtin pile-up
Nature Chemical Biology, 2010Aggregation of huntingtin protein with an expanded polyglutamine region is enhanced by its 17-residue N-terminal domain, which binds to itself and to the polyglutamine region. This enhancement is inhibited when the N-terminal domain binds to the chaperonin TRiC.
Susan W, Liebman, Stephen C, Meredith
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Huntingtin Polyglutamine-Dependent Protein Aggregation in Reconstituted Cells
ACS Synthetic Biology, 2017One of the aims of synthetic biology is bottom-up construction of reconstituted human cells for medical uses. To that end, we generated giant unilamellar vesicles (GUVs) that contained a HeLa cell extract, which comprises a cell-free protein synthesis (CFPS) system.
Kodai Machida +5 more
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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–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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Detecting mutant huntingtin protein in HD patients
Science Translational Medicine, 2015Mutant huntingtin protein in the cerebrospinal fluid of patients with Huntington’s disease can seed prion-like protein propagation.
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The contemporary management of cancers of the sinonasal tract in adults
Ca-A Cancer Journal for Clinicians, 2023Rajat Thawani
exaly
[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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