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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 25 Jan 2016.

Huntingtin

huntingtin
Top mentioned proteins: CAN, AGE, ACID, V1a, HAD
Papers using huntingtin antibodies
A comparison of normalization methods for high density oligonucleotide array data based on variance and bias
Supplier
Flotte Terence R et al., In Molecular Therapy, 2002
... Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice ...
Modulation of the in situ activity of tissue transglutaminase by calcium and GTP
Supplier
Johnson Gail V.W. et al., In The Journal of Cell Biology, 1997
... The BamHI–XhoI huntingtin cDNA fragments were also subcloned into the Amersham Pharmacia Biotech and XhoI sites of the pECFP-N1 vector (CLONTECH Laboratories, Inc.) (N-Q18) (pECFP-N1-18Q) ...
Papers on huntingtin
Increased Olfactory Bulb BDNF Expression Does Not Rescue Deficits in Olfactory Neurogenesis in the Huntington's Disease R6/2 Mouse.
New
Guthrie et al., Boca Raton, United States. In Chem Senses, 18 Feb 2016
UNASSIGNED: Huntington's disease (HD) is an inherited neurodegenerative disorder caused by expansion of CAG trinucleotide repeats in the huntingtin gene.
Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA.
New
Impact
Hipp et al., Martinsried, Germany. In Science, 08 Feb 2016
Here, we analyzed the compartment specificity of aggregate toxicity using artificial β-sheet proteins, as well as fragments of mutant huntingtin and TAR DNA binding protein-43 (TDP-43).
Huntington's Disease: Relationship Between Phenotype and Genotype.
New
Wu et al., Shanghai, China. In Mol Neurobiol, 07 Feb 2016
It is caused by the dynamic mutation in CAG triplet repeat number in exon 1 of huntingtin (HTT) gene.
PPAR-δ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically.
New
Impact
La Spada et al., San Diego, United States. In Nat Med, 31 Jan 2016
Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the huntingtin (HTT) gene, which encodes a polyglutamine tract in the HTT protein.
CAMELOT: A machine learning approach for coarse-grained simulations of aggregation of block-copolymeric protein sequences.
New
Pappu et al., Saint Louis, United States. In J Chem Phys, 28 Jan 2016
We demonstrate the utility of our coarse-graining approach using the block-copolymeric sequence from the exon 1 encoded sequence of the huntingtin protein.
Widespread CNS gene transfer and silencing after systemic delivery of novel AAV-AS vector.
New
Sena-Esteves et al., Auburn, United States. In Mol Ther, 28 Jan 2016
A single intravenous injection of an AAV-AS vector encoding an artificial microRNA targeting huntingtin (Htt) resulted in 33-50% knockdown of Htt across multiple CNS structures in adult mice.
Biased Type 1 Cannabinoid Receptor Signalling Influences Neuronal Viability in a Cell Culture Model of Huntington Disease.
New
Denovan-Wright et al., In Mol Pharmacol, 23 Jan 2016
UNASSIGNED: Huntington disease (HD) is an inherited, autosomal dominant, neurodegenerative disorder with limited treatment options.
Impaired development of cortico-striatal synaptic connectivity in a cell culture model of Huntington's disease.
New
Raymond et al., Vancouver, Canada. In Neurobiol Dis, 19 Jan 2016
UNASSIGNED: Huntington's disease (HD) is a genetically inherited neurodegenerative disease caused by a mutation in the gene encoding the huntingtin protein.
AMPK activation protects from neuronal dysfunction and vulnerability across nematode, cellular and mouse models of Huntington's disease.
New
Neri et al., Paris, France. In Hum Mol Genet, 17 Jan 2016
Genetic and pharmacological manipulation of aak-2/AMPKα shows that AMPK activation protects C. elegans neurons from the dysfunction induced by human exon-1 huntingtin (Htt) expression, in a daf-16/FOXO dependent manner.
α-Synuclein and huntingtin exon 1 amyloid fibrils bind laterally to the cellular membrane.
New
Melki et al., Gif-sur-Yvette, France. In Sci Rep, 31 Dec 2015
By characterizing the binding properties of aggregates made of α-synuclein or huntingtin exon 1 protein displaying similar composition and structure but different lengths to mammalian cells we demonstrate that in both cases aggregates bind laterally to the cellular membrane, with aggregates extremities displaying little or no role in membrane binding.
Safety, tolerability, and efficacy of PBT2 in Huntington's disease: a phase 2, randomised, double-blind, placebo-controlled trial.
Impact
Huntington Study Group Reach2HD Investigators, In Lancet Neurol, 2015
BACKGROUND: PBT2 is a metal protein-attenuating compound that might reduce metal-induced aggregation of mutant huntingtin and has prolonged survival in a mouse model of Huntington's disease.
Reduced Levels of Proteasome Products in a Mouse Striatal Cell Model of Huntington's Disease.
Fricker et al., São Vicente, Brazil. In Plos One, 2014
Huntington's disease is the result of a long polyglutamine tract in the gene encoding huntingtin protein, which in turn causes a large number of cellular changes and ultimately results in neurodegeneration of striatal neurons.
The Neuropathology of Huntington´s disease: classical findings, recent developments and correlation to functional neuroanatomy.
Korf et al., Frankfurt am Main, Germany. In Adv Anat Embryol Cell Biol, 2014
which encodes the mutated protein huntingtin (Htt).
Activation and regulation of caspase-6 and its role in neurodegenerative diseases.
Impact
Su et al., Key West, United States. In Annu Rev Pharmacol Toxicol, 2014
Cleavage at the caspase-6 site in mutated huntingtin protein is a prerequisite for the development of the characteristic behavioral and neuropathological features of Huntington's disease.
Targeting Hsp90/Hsp70-based protein quality control for treatment of adult onset neurodegenerative diseases.
Impact
Lieberman et al., In Annu Rev Pharmacol Toxicol, 2014
Critical target proteins that unfold and aggregate in these diseases, such as the polyglutamine androgen receptor in spinal and bulbar muscular atrophy, huntingtin in Huntington's disease, α-synuclein in Parkinson's disease, and tau in Alzheimer's disease, are client proteins of heat shock protein 90 (Hsp90), and their turnover is regulated by the protein quality control function of the Hsp90/Hsp70-based chaperone machinery.
Huntingtin is required for mitotic spindle orientation and mammalian neurogenesis.
GeneRIF
Humbert et al., Orsay, France. In Neuron, 2010
The specific disruption of Drosophila huntingtin in neuroblast precursors leads to spindle misorientation; Drosophila huntingtin restores spindle misorientation in mammalian cells.
A genomewide RNA interference screen for modifiers of aggregates formation by mutant Huntingtin in Drosophila.
GeneRIF
Perrimon et al., Boston, United States. In Genetics, 2010
a genomewide RNA interference screen for regulators of mutant Htt aggregation
Inactivation of Drosophila Huntingtin affects long-term adult functioning and the pathogenesis of a Huntington's disease model.
GeneRIF
Perrimon et al., Boston, United States. In Dis Model Mech, 2009
dHtt is required for maintaining the mobility and long-term survival of adult animals, and for modulating axonal terminal complexity in the adult brain.
Glial cell lineage expression of mutant ataxin-1 and huntingtin induces developmental and late-onset neuronal pathologies in Drosophila models.
GeneRIF
Okazawa et al., Tokyo, Japan. In Plos One, 2008
mutant ataxin-1 and huntingtin induce developmental and late-onset neuronal pathologies in Drosophila models
RNAi screening in Drosophila cells identifies new modifiers of mutant huntingtin aggregation.
GeneRIF
Nukina et al., Wako, Japan. In Plos One, 2008
genes related to nuclear transport, nucleotide processes, and signaling are modifiers of huntingtin aggregation
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