Huntingtin Protein

A protein that is highly expressed in the nervous system as well as other tissues; its size and structure vary due to polymorphisms. Expanded CAG TRINUCLEOTIDE REPEATS have been identified in the Huntingtin (HD) Gene of patients with HUNTINGTON DISEASE and are associated with abnormal PROTEIN AGGREGATES. Huntingtin interacts with proteins involved in a variety of gene expression and cellular processes; it is also essential for embryonic development.

Also Known As:

Networked: 542 relevant articles (7 outcomes, 21 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Hayden, Michael R: 17 articles (08/2022 - 08/2002)
2.	Leavitt, Blair R: 14 articles (01/2022 - 08/2002)
3.	Bates, Gillian P: 12 articles (07/2022 - 05/2007)
4.	Tabrizi, Sarah J: 12 articles (01/2022 - 05/2007)
5.	Lashuel, Hilal A: 10 articles (01/2022 - 02/2014)
6.	Wild, Edward J: 10 articles (01/2022 - 05/2010)
7.	Weiss, Andreas: 9 articles (11/2018 - 12/2009)
8.	Hannan, Anthony J: 9 articles (01/2018 - 03/2004)
9.	Marsh, J Lawrence: 8 articles (01/2021 - 04/2002)
10.	Ross, Christopher A: 8 articles (01/2019 - 03/2003)

Related Diseases

1.	Huntington Disease (Huntington's Disease) 06/01/2009 - "Use of RNA interference to reduce huntingtin protein (htt) expression in affected brain regions may provide an effective treatment for Huntington disease (HD), but it remains uncertain whether suppression of both wild-type and mutant alleles in a heterozygous patient will provide more benefit than harm. " 11/01/2023 - "This study sought to quantify the density of mutant huntingtin protein and neuroinflammatory glial changes in the midcingulate cortex of postmortem patients with Huntington's disease and determine if either correlates with the presentation of mood, motor, or mixed symptomatology. " 01/01/2023 - "In a previous study, we have shown that parabiotic coupling of a knock-in mouse model (zQ175) of Huntington's disease (HD) to wild-type (WT) littermates resulted in a worsening of the normal phenotype as seen by detection of mutant huntingtin protein (mHTT) aggregates within peripheral organs and the cerebral cortex as well as vascular abnormalities in WT mice. " 04/27/2022 - "Fighting the Huntington's Disease with a G-Quadruplex-Forming Aptamer Specifically Binding to Mutant Huntingtin Protein: Biophysical Characterization, In Vitro and In Vivo Studies." 01/01/2018 - "On December 11 of 2017, Ionis Pharmaceuticals published a press release announcing dose-dependent reductions of mutant huntingtin protein in their HTTRx Phase 1/2a study in Huntington disease (HD) patients. "
2.	Neurodegenerative Diseases (Neurodegenerative Disease) 01/01/2020 - "Unlike most neurodegenerative disorders, HD is caused by a single genetic mutation resulting in a CAG repeat expansion and the production of a mutant Huntingtin protein (mHTT). " 04/01/1998 - "Huntingtin protein colocalizes with lesions of neurodegenerative diseases: An investigation in Huntington's, Alzheimer's, and Pick's diseases." 11/20/2023 - "Huntington's disease (HD) is a devastating neurodegenerative disorder characterized by the accumulation of mutant Huntingtin protein (mHTT) and oxidative stress-induced neuronal damage. " 10/04/2023 - "Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a CAG repeat expansion in the HD gene, coding for huntingtin protein (HTT). " 12/06/2021 - "Huntington Disease (HD) is a dominant, lethal neurodegenerative disorder caused by the abnormal expansion (>35 copies) of a CAG triplet located in exon 1 of the HTT gene encoding the huntingtin protein (Htt). "
3.	Parkinson Disease (Parkinson's Disease) 01/01/2023 - "Regarding Parkinson's disease (PD), another example of ND, the bioactive compounds from Holothuria scabra and Xylaria sp., showed to be able to reduce the degeneration of dopaminergic neurons, reduce the deposition of alpha synuclein and reduce the formation of Mutant Huntingtin protein (Mhtt). " 11/08/2016 - "In this study, we firstly showed that an oligomer-specific single-chain variable fragment antibody, W20 simultaneously improved motor and cognitive function in Parkinson's disease and Huntington's disease mouse models, and attenuated a number of neuropathological features by reducing α-synuclein and mutant huntingtin protein aggregate load and preventing synaptic degeneration. " 10/24/2019 - "In this review, using cases of Alzheimer's disease (amyloid-beta peptide), Parkinson's disease (alpha-synuclein peptide) and Huntington's disease (huntingtin protein), we illustrate existing challenges in experimental investigations and summarize recent relevant numerical simulation studies into amyloidogenic peptide-membrane interactions. " 03/06/2023 - "The soluble-to-toxic transformation of intrinsically disordered amyloidogenic proteins such as amyloid beta (Aβ), α-synuclein, mutant Huntingtin Protein (mHTT) and islet amyloid polypeptide (IAPP) among others is associated with disorders such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD) and Type 2 Diabetes (T2D), respectively. " 10/01/2014 - "In lipid rafts, interactions of glycosphingolipids, including ganglioside, with proteins may be responsible for the misfolding events that cause the fibril and/or aggregate processing of disease-specific proteins, such as α-synuclein, in Parkinson's disease, huntingtin protein in Huntington's disease, and copper-zinc superoxide dismutase in amyotrophic lateral sclerosis. "
4.	Inflammation (Inflammations) 05/01/2012 - "The mutant huntingtin protein causes neuronal dysfunction and eventual cell death in which transcriptional impairment, excitotoxicity, oxidative damage, inflammation, apoptosis and mitochondrial dysfunction are all implicated. " 01/01/2022 - "Potential interventions for Huntington's disease include therapies targeting huntingtin DNA and RNA, clearance of huntingtin protein, DNA repair pathways, and other treatment strategies targeting inflammation and cell replacement. "
5.	Infections 11/01/2016 - "chaffeensis infection and induced autophagosome formation, cleared an aggregation-prone mutant huntingtin protein in a class III PtdIns3K-dependent manner, and enhanced ehrlichial proliferation. "

Related Drugs and Biologics

1.	Synucleins
2.	Single-Chain Antibodies
3.	DNA (Deoxyribonucleic Acid)
4.	Actin Depolymerizing Factors (Cofilins)
5.	Brain-Derived Neurotrophic Factor (BDNF)
6.	Intravenous Immunoglobulins (IVIG)
7.	RNA (Ribonucleic Acid)
8.	polyglutamine
9.	Proteins (Proteins, Gene)
10.	Protein Aggregates

Related Therapies and Procedures

1.	Therapeutics