Huntington 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. The huntingtin protein is required ...
Huntington disease: Transneuronal propagation of mutant huntingtin proteinMutant huntingtin (mHTT) might spread from neuron to neuron in a prion-like fashion. Pecho-Vrieseling et al . discovered that neurons derived from human stem cells could acquire mHTT aggregates and HD-like pathology when ...
ARTICLES Study finds potential entry points to Huntington's therapy March 1, 2022 ByJohn Fox No Comments A Chinese study led by researchers at Fudan University in Shanghai has described a novel strategy to target the "undruggable" mutant Huntingtin protein by screening for compounds that directly ...
Detection of high levels of mutant huntingtin protein in tear fluid of patients with Huntington's Disease Purpose : Huntington's disease (HD) is an autosomal dominant, fully penetrant, neurodegenerative disease that most commonly affects adults in mid-life. HD is caused by a CAG repeat expansion...
Huntington’s disease (HD) is a rare neurodegenerative disorder caused by an expansion of the CAG trinucleotide repeat in the huntingtin gene which encodes the mutant huntingtin protein (mHTT) that is associated with HD-related neuropathophysiology. Noninvasive visualization of mHTT aggregates in the...
The polyglutamine expansion of huntingtin (mHTT) causes Huntington disease (HD) and neurodegeneration, but the mechanisms remain unclear. Here, we found that mHtt promotes ribosome stalling and suppresses protein synthesis in mouse HD striatal neuronal cells. Depletion of mHtt enhances protein synthesis...
Huntington's disease is an autosomal-dominant neurodegenerative disorder, with chorea as the most prominent manifestation. The disease is caused by abnormal expansion of CAG codon repeats in theIT15gene, which leads to the expression of a glutamine-rich protein named mutant Huntingtin (Htt). Because...
Huntington's disease (HD) is caused by a CAG repeaBo Wang a b cLi Zeng a dSean A. Merillat aSvetlana Fischer aJoseph Ochaba e fLeslie M. Thompson e fSami J. Barmada aKenneth M. Scaglione gHenry L. Paulson a bNeurobiology of Disease...
Huntingtin (htt) is a multi-domain protein of 350 kDa that is mutated in Huntington's disease (HD) but whose function is yet to be fully understood. This absence of information is due in part to the difficulty of manipulating large DNA fragments by using conventional molecular cloning techniqu...
Sari Y: Huntington's Disease: From Mutant Huntingtin Protein to Neurotrophic Factor Therapy Int J Biomed Sci. 2011 7/2:89-100Y. Sari, "Huntington's disease: from mutant huntingtin pro- tein to neurotrophic factor therapy," International Journal of Biomedical Sciences, vol. 7, no. 2, pp. ...