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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 30 Oct 2014.

Huntingtin

huntingtin
Top mentioned proteins: CAN, AGE, ACID, V1a, Brain-derived neurotrophic factor
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
Altered Ca2+ signaling in skeletal muscle fibers of the R6/2 mouse, a model of Huntington's disease.
New
Melzer et al., Ulm, Germany. In J Gen Physiol, 30 Nov 2014
Huntington's disease (HD) is caused by an expanded CAG trinucleotide repeat within the gene encoding the protein huntingtin.
Aged Monkey Brains Reveal the Role of Ubiquitin-Conjugating Enzyme UBE2N in the Synaptosomal Accumulation of Mutant Huntingtin.
New
Li et al., Beijing, China. In Hum Mol Genet, 24 Nov 2014
UNLABELLED: Although misfolded proteins are ubiquitinated and cleared by the proteasome, they can accumulate in synapses in aged neurons to promote synaptic dysfunction in a variety of neurodegenerative diseases, including Huntington's disease (HD), which is caused by polyglutamine expansion in huntingtin.
Polyglutamine Aggregation in Huntington Disease: Does Structure Determine Toxicity?
New
Djian et al., Paris, France. In Mol Neurobiol, 22 Nov 2014
The mutational expansion of polyglutamine beyond a critical length produces a toxic gain of function in huntingtin and results in neuronal death.
Single nucleotide seed modification restores in vivo tolerability of a toxic artificial miRNA sequence in the mouse brain.
New
Davidson et al., Philadelphia, United States. In Nucleic Acids Res, 20 Nov 2014
UNLABELLED: Huntington's disease is a fatal neurodegenerative disease caused by polyglutamine-expansion in huntingtin (HTT).
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, 01 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, 25 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, 09 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.
Cystathionine γ-lyase deficiency mediates neurodegeneration in Huntington's disease.
New
Impact
Snyder et al., Baltimore, United States. In Nature, Jun 2014
Huntington's disease is an autosomal dominant disease associated with a mutation in the gene encoding huntingtin (Htt) leading to expanded polyglutamine repeats of mutant Htt (mHtt) that elicit oxidative stress, neurotoxicity, and motor and behavioural changes.
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.
Ubiquitin-proteasome system involvement in Huntington's disease.
Review
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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
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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.
Proteostasis in striatal cells and selective neurodegeneration in Huntington's disease.
Review
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Finkbeiner et al., San Francisco, United States. In Front Cell Neurosci, Dec 2013
Although mutant huntingtin, the protein responsible for HD, is expressed ubiquitously, a subpopulation of neurons in the striatum is the first to succumb.
Skeletal muscle pathology in Huntington's disease.
Review
New
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).
Huntingtin promotes mTORC1 signaling in the pathogenesis of Huntington's disease.
New
Subramaniam et al., Jupiter, United States. In Sci Signal, Dec 2013
In patients with Huntington's disease (HD), the protein huntingtin (Htt) has an expanded polyglutamine (poly-Q) tract.
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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