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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 10 Dec 2014.

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
Huntington Disease: Pathogenesis and Treatment.
Review
New
Albin et al., Ann Arbor, United States. In Neurol Clin, 28 Feb 2015
UNLABELLED: Huntington disease (HD) is an autosomal dominant inherited neurodegenerative disease characterized by progressive motor, behavioral, and cognitive decline, culminating in death.
Overexpression of Q-rich prion-like proteins suppresses polyQ cytotoxicity and alters the polyQ interactome.
New
Hipp et al., Martinsried, Germany. In Proc Natl Acad Sci U S A, 08 Jan 2015
This system allowed us to screen for genes that when overexpressed reduce the toxic effects of an N-terminal fragment of mutant huntingtin with 103 Q. Surprisingly, among the identified suppressors were three proteins with Q-rich, prion-like domains (PrDs): glycine threonine serine repeat protein (Gts1p), nuclear polyadenylated RNA-binding protein 3, and minichromosome maintenance protein 1. Overexpression of the PrD of Gts1p, containing an imperfect 28 residue glutamine-alanine repeat, was sufficient for suppression of toxicity.
Huntingtin interacting proteins 14 and 14-like are required for chorioallantoic fusion during early placental development.
New
Hoodless et al., Vancouver, Canada. In Dev Biol, 02 Jan 2015
UNLABELLED: Huntington disease (HD) is an adult-onset neurodegenerative disease characterized by motor, cognitive, and psychiatric symptoms that is caused by a CAG expansion in the HTT gene.
In vitro ischemia decreases histone H4K16 Acetylation in neural cells.
New
Papkovsky et al., Cork, Ireland. In Febs Lett, 02 Jan 2015
A decrease in H4K16Ac levels can affect the expression of mitochondrial uncoupling protein 2 (UCP2), huntingtin-interacting protein 1 (HIP1) and Notch-pathway genes in a cell-specific manner.
Safety, tolerability, and efficacy of PBT2 in Huntington's disease: a phase 2, randomised, double-blind, placebo-controlled trial.
New
Impact
Huntington Study Group Reach2HD Investigators, In Lancet Neurol, 14 Dec 2014
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.
Mutations in the ubiquitin-binding domain of OPTN/optineurin interfere with autophagy-mediated degradation of misfolded proteins by a dominant-negative mechanism.
New
Tu et al., In Autophagy, 30 Nov 2014
Here, we demonstrated that OPTN colocalized with inclusion bodies (IBs) formed by mutant HTT/huntingtin protein (mHTT) in R6/2 transgenic mice and IBs formed by 81QNmHTT (nuclear form), 109QmHTT (cytoplasmic form) or the truncated form of TARDBP/TDP-43 (TARDBPND251) in Neuro2A cells.
Activation and Regulation of Caspase-6 and Its Role in Neurodegenerative Diseases.
New
Impact
Su et al., Beijing, China. In Annu Rev Pharmacol Toxicol, 17 Nov 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.
Autophagy in Huntington disease and huntingtin in autophagy.
Review
New
Hayden et al., Vancouver, Canada. In Trends Neurosci, Nov 2014
In Huntington disease (HD), an expansion of the polyglutamine (polyQ) tract in the N-terminus of the huntingtin (HTT) protein leads to protein aggregation.
Targeting Hsp90/Hsp70-Based Protein Quality Control for Treatment of Adult Onset Neurodegenerative Diseases.
New
Impact
Lieberman et al., Ann Arbor, United States. In Annu Rev Pharmacol Toxicol, Oct 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.
Slowing of neurodegeneration in Parkinson's disease and Huntington's disease: future therapeutic perspectives.
Review
New
Impact
Bezard et al., New York City, United States. In Lancet, Sep 2014
In Huntington's disease, strategies might also be directed at mitochondrial bioenergetics and turnover, the prevention of protein dysregulation, disruption of the interaction between huntingtin and p53 or huntingtin-interacting protein 1 to reduce apoptosis, and interference with expression of mutant huntingtin at both the nucleic acid and protein levels.
Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease.
New
Impact
Yang et al., Los Angeles, United States. In Nat Med, May 2014
Huntington's disease (HD) is a fatal dominantly inherited neurodegenerative disorder caused by a CAG repeat expansion leading to an elongated polyglutamine stretch in huntingtin.
Early Retinal Function Deficit without Prominent Morphological Changes in the R6/2 Mouse Model of Huntington's Disease.
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Kalesnykas et al., Kuopio, Finland. In Plos One, Dec 2013
Huntington's disease (HD) is an inherited neurodegenerative disorder that primarily affects the medium-size GABAergic neurons of striatum.
Skeletal muscle pathology in Huntington's disease.
Review
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Mielcarek et al., Poznań, Poland. In Front Physiol, Dec 2013
Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by the expansion of a polyglutamine stretch within the huntingtin protein (HTT).
Ubiquitin-proteasome system involvement in Huntington's disease.
Review
New
Lucas et al., Madrid, Spain. In Front Mol Neurosci, Dec 2013
Huntington's disease (HD) is a genetic autosomal dominant neurodegenerative disease caused by the expansion of a CAG repeat in the huntingtin (htt) gene.
Possible involvement of self-defense mechanisms in the preferential vulnerability of the striatum in Huntington's disease.
Review
New
Brouillet et al., Fontenay-aux-Roses, France. In Front Cell Neurosci, Dec 2013
HD is caused by a mutation in the huntingtin gene that consists in a CAG repeat expansion translated into an abnormal poly-glutamine (polyQ) tract in the huntingtin (Htt) protein.
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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