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Topic: Huntingtin

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	HD and huntingtin: 1998
		The gene encodes the protein huntingtin with a polyglutamine tract encoded by the CAG repeat at the N-terminus.
		Therefore, the primary defect in the absence of huntingtin lies in extraembryonic tissues, whereas the epiblast and its derivatives are affected secondarily.
		Consistent with preliminary histochemical analysis indicating that at least the transport of ferric ions is defective in Hdh mutants and in conjunction with the known localization of huntingtin in the membranes of vesicles associated with microtubules, we hypothesize that this protein is involved in the intracellular trafficking of nutrients in early embryos.
	lansbury.bwh.harvard.edu /Literature/Review/hd_and_huntingtin_1998.htm (9938 words)

	HD and huntingtin: 2002
		Huntingtin is particularly abundant in the perinuclear region, where it is also associated with microtubules and in the centrosomal region, where it co-localizes with gamma-tubulin.
		Since the perinuclear concentration of huntingtin does not depend on the number of its glutamine repeats, we propose that inclusions are found in perinuclear and intranuclear locations because the beta-tubulin binding property of huntingtin brings it to the perinuclear region, from which it readily gains access to the nucleus.
		Huntington's Disease (HD) is an inherited neurodegenerative disease caused by expansion of a polyglutamine tract in the huntingtin protein.
	lansbury.bwh.harvard.edu /literature/review/hd_and_huntingtin_2002.htm (6164 words)

	huntingtin - humpath.com - a website dedicated to human pathology. (Site not responding. Last check: 2007-11-05)
		The reason for this is that the initial aggregates are efficiently removed by cellular chaperones and it is only when these become overwhelmed, after many years, that the aggregates develop and with them the clinical manifestations.
		The cause of the aggregation is the presence in the tail of the huntingtin molecule of a large glutamine-repeat domain that can undergo an inheritable extension.
		This model suggests that the glutamine repeats form a cylindrical sheet made up of beta-strands with 20 residues per helical turn, and provides a satisfying hypothesis for the crucial threshold of 37-40 glutamine repeats that are required for neurodegeneration in Huntington disease.
	www.humpath.com /article.php3?id_article=647 (247 words)

	huntingtin definition - Medical Dictionary definitions of popular medical terms
		Huntingtin: The name of the gene responsible for Huntington disease (HD) and of the protein encoded by that gene.
		The molecular basis of HD is an increase in the length of a CAG triplet repeat (or 'polyglutamine') within the huntingtin gene.
		The huntingtin mutation (gene change) is fully dominant; homozygotes (who have two copies of the huntingtin gene) are no more severely affected than heterozygotes (who have only one copy of the gene).
	www.medterms.com /script/main/art.asp?articlekey=11563 (230 words)

	BioMed Central \| Full text \| Huntingtin gene evolution in Chordata and its peculiar features in the ascidian Cionagenus
		Huntingtin is a large protein (> 3000 amino acids long in vertebrates), characterized in humans by the presence of an amino-terminal polymorphic polyglutamine (polyQ) tract whose aberrant expansion causes Huntington's Disease (HD), a progressive neurodegenerative disease accompanied by neuronal dysfunction and cell loss in the brain [1].
		The huntingtin gene or transcript has been characterized in a number of vertebrate and invertebrate species [10-17], and it seems to be absent in yeast and lower eukaryotes [16].
		Although in huntingtin there is no indication of a correlation between the boundaries of HEAT domains and their coding exons, we can not exclude the existence of a correlation between the structure of the gene and the unknown domain structure of the protein.
	www.biomedcentral.com /1471-2164/7/288 (8094 words)

	HOPES Glossary, H
		A cytoplasmic protein (associated with the membrane cytoskeleton) that is expressed predominantly in the brain.
		Huntingtin proteins with an expanded number of glutamines show decreased binding to HIP-1.
		Hsp 40 - The mouse analog of HDJ1.
	www.stanford.edu /group/hopes/sttools/gloss/h.html (1911 words)

	New Antibody Panel to Huntingtin
		Huntingtin is a 350kDa protein of 3144 amino acids that is altered in Huntington's disease (HD).
		The abnormal form of Huntingtin aggregates in vitro and forms neuronal intranuclear and cytoplasmic inclusions in HD patients.
		The expanded polyglutamine repeats formed by this mutation of the Huntingtin gene have been proposed to cause neuronal degeneration in HD through abnormal interactions with other proteins containing short polyglutamine tracts such as the CREB binding protein (CBP), a protein that promotes cell survival and is a major mediator of survival signals in mature neurons.
	www.acris-antibodies.com /focus_review/focus0011-Huntingtin.php (347 words)

	Researchers shed new light on the function of huntingtin, the protein whose mutation underlies Huntington’s disease (Site not responding. Last check: 2007-11-05)
		CNRS and Inserm research scientists at the Institut Curie have shed new light on the function of huntingtin, the protein whose mutation underlies Huntington's disease.
		But when it is mutated, the reverse happens: by a mechanism as yet unelucidated, the mutation in huntingtin leads to accelerated death of neurons in the striatum, the brain region where Huntington's disease arises.
		In its normal state, huntingtin "boosts" the transport of BDNF(3), which is needed for survival of neurons in the striatum.
	www.news-medical.net /?id=3158 (689 words)

	Tissue Transglutaminase Does Not Contribute to the Formation of Mutant Huntingtin Aggregates -- Chun et al. 153 (1): 25 ...
		In the N-Q18 cells, huntingtin immunostaining was diffuse throughout the cytoplasm (a).
		Huntingtin was immunoprecipitated with the polyclonal huntingtin antibody and immunoblotted with a monoclonal tTG antibody (left).
		huntingtin is an in vitro substrate of tTG (
	www.jcb.org /cgi/content/full/153/1/25 (5482 words)

	Perinuclear localization of huntingtin as a consequence of its binding to microtubules through an interaction with ...
		Huntingtin and MAP-2 cosediment with microtubules, whereas actin and caspase-1, two proteins not known to be associated with microtubules, remained virtually entirely in the supernatant.
		Huntingtin is found at the centrosome in a fraction of the cell population.
		Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons.
	jcs.biologists.org /cgi/content/full/115/5/941 (4903 words)

	HD - huntingtin (Huntington disease) - Genetics Home Reference
		Although the exact function of this protein is unknown, it appears to play an important role in nerve cells and is essential for normal development before birth.
		Huntingtin is found in many of the body's tissues, with the highest levels of activity in the brain.
		Within cells, this protein may be involved in signaling, transporting materials, attaching (binding) to proteins and other structures, and protecting the cell from self-destruction (apoptosis).
	ghr.nlm.nih.gov /gene=hd (753 words)

	The Influence of Huntingtin Protein Size on Nuclear Localization and Cellular Toxicity -- Hackam et al. 141 (5): 1097 ...
		Huntingtin located in the nucleus appears pink as the red stain is overlaid with blue.
		Huntingtin containing 15 repeats does not form aggregates but can be localized in the nucleus depending on the protein size.
		Huntingtin is localized entirely in the nucleus in the 436 and 771 constructs in the cells shown.
	www.jcb.org /cgi/content/full/141/5/1097 (6179 words)

	Huntingtin (human) (Site not responding. Last check: 2007-11-05)
		This is thought to be caused by an expanded, unstable trinucleotide repeat in the Huntingtin gene, which translates as a polyglutamine repeat in the protein product.
		The Huntingtin locus is large, spanning 180 kb and consisting of 67 exons.
		The Huntingtin gene is widely expressed and is required for normal development.
	www.phosphosite.org /ParentProteinHomePage.jsp?organismId=5&proteinId=1643 (191 words)

	WE MOVE - Huntington's Disease - Mutant Huntingtin Protein and Intracellular Abnormalities
		However, the disease processes associated with HD are thought to be caused by a toxic "gain of function," meaning that the mutated gene's protein (mutant huntingtin) interferes with normal cellular functioning.
		In addition, researchers indicate that expansion of the amino acid glutamine within the huntingtin protein is associated with accumulations or inclusions of mutant huntingtin and an associated protein (ubiquitin) within the nucleus of neurons in the basal ganglia (i.e., striatum) and cerebral cortex.
		In addition, investigators have demonstrated that caspase-1, an enzyme involved in controlling cellular death, is activated in the brain in patients with HD and a transgenic mouse model of the disease.
	www.wemove.org /hd/hd_path_mut.html (224 words)

	The Jan Braun Laboratory
		Huntingtin is a widely expressed 350 kDa cytosolic protein bearing an N-terminal polyglutamine tract that is is proteolytically cleaved by calpains and caspases.
		The resultant huntingtin fragments may be biologically active; however, the precise function(s) of huntingtin and/or the huntingtin fragments remains enigmatic.
		Defining the biological role of huntingtin and huntingtin fragments (native and expanded) is a current focus of our laboratory.
	www.ucalgary.ca /~braunj/projects.html (495 words)

	Dysfunction of Wild-Type Huntingtin in Huntington disease -- Cattaneo 18 (1): 34 -- Physiology
		indicate that huntingtin is a substrate for caspases.
		Some of the activities of huntingtin and its interactions with key cellular elements involved in apoptotic cascades are summarized.
		Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract.
	physiologyonline.physiology.org /cgi/content/full/18/1/34 (2474 words)

	Accumulation of Mutant Huntingtin Fragments in Aggresome-like Inclusion Bodies as a Result of Insufficient Protein ...
		Top, misfolded proteins, including N-terminal huntingtin fragments with 20 glutamines (HDQ20; blue) are ubiquitinated (small red circles) and degraded in a hypothetical organelle, the degrasome, containing the proteasomal subcomplexes 11S, 19S, and 20S and the heat shock proteins Hsp40 and Hsp70.
		Charles, V., Mezey, E., Reddy, P.H., Dehejia, A., Young, T.A., Polymeropoulos, M.H., Brownstein, M.J., and Tagle, D.A. Alpha-synuclein immunoreactivity of huntingtin polyglutamine aggregates in striatum and cortex of Huntington's disease patients and transgenic mouse models.
		Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates.
	www.molbiolcell.org /cgi/content/full/12/5/1393 (7775 words)

	Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis -- Li et al. 9 (19): 2859 -- Human ... (Site not responding. Last check: 2007-11-05)
		Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis -- Li et al.
		Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis
		huntingtin induces the activation of caspase-3 and the release
	hmg.oxfordjournals.org /cgi/content/abstract/9/19/2859 (636 words)

	Inhibition of Calcineurin by FK506 Protects against Polyglutamine-Huntingtin Toxicity through an Increase of Huntingtin ...
		of endogenous huntingtin is a physiological substrate of calcineurin.
		Huntingtin colocalizes with calcineurin and is dephosphorylated during Ca -induced calcineurin activation.
		B, Huntingtin colocalized with the catalytic subunit of calcineurin (CaNA) to vesicular structures.
	www.jneurosci.org /cgi/content/full/26/5/1635 (6753 words)

	Mutant Huntingtin Causes Context-Dependent Neurodegeneration in Mice with Huntington's Disease -- Yu et al. 23 (6): ...
		This antibody reacted with diffuse huntingtin in the
		In addition to protein context, the toxicity of mutant huntingtin fragments is also determined by a number of variables.
		Huntingtin phosphorylation on serine 421 is significantly reduced in the striatum and by polyglutamine expansion in vivo
	www.jneurosci.org /cgi/content/full/23/6/2193 (7158 words)

	InterPro: IPR000091 Huntingtin
		Huntingtin normally contains 10-35 repeats, but shows 36-120 repeats in the disease form.
		Migration differences between normal and mutated huntingtin in a denaturing polyacrylamide gel suggest that the poly-Gln stretch disrupts the protein conformation.
		This finding is consistent with the observation that Gln repeats may form tightly-linked beta-sheets that could act as polar zippers [ 2 ].
	www.ebi.ac.uk /interpro/IEntry?ac=IPR000091 (3103 words)

	Mutant huntingtin goes straight to the heart - Nature Neuroscience
		(The mitochondria from HD patients were derived from lymphoblasts, because huntingtin is ubiquitously expressed, and these cells can be easily obtained from blood.) In another experiment, the authors reproduced the mitochondrial changes observed in patients and mutant mice by directly applying (in vitro) a small amino-terminal portion of mutant huntingtin to mitochondria from normal subjects.
		In addition, mutant huntingtin at the mitochondrial membrane seems to cause abnormal opening of permeability transition pores (PTPs); these structures are located on mitochondrial membranes, and calcium moves through them.
		Finally, calcium mediates cytoplasmic and nuclear events (caspase and calpaine activation, loss of wild-type huntingtin, transcription factors sequestration, histone acetylation, changes in gene transcription), as well as membrane events (neuronal depolarization, increased sensitivity to the toxic effects of NMDA and dopamine), leading to cell death.
	www.nature.com /cgi-taf/DynaPage.taf?file=/neuro/journal/v5/n8/full/nn0802-711.html (1161 words)

	HIP1 - huntingtin interacting protein 1
		Huntingtin interacting protein 1 is a membrane-associated protein that colocalizes with huntingtin.
		In chronic myelomonocytic leukemia, this gene has been found to be fused with platelet-derived growth factor-beta receptor gene and HIP1 has been implicated in the pathogenesis of hematopoietic malignancies.
		The huntingtin interacting protein 1 gene is thought to encode a 914 amino acid protein but may encode a longer protein due to another translation initiation site further upstream.
	www.ucsf.edu /pgdb/gene/50.html (205 words)

	huntingtin Search Results From Healthline
		It is the terrible disease that caused the decline and death of the American folksinger Woody Guthrie (1912-1967) who wrote "Your Land is My Land", "So Long it's Been Good to Know You" and many other memorable songs.
		Immunohistochemical analysis with DARPP-32 antibody were performed, and the size of the lesions were determined for all constructs and...
		Huntington's Associated Protein-1 or HAP-1, became known by the company it keeps: it's the first protein ever found that chemically binds to huntingtin, the protein produced by the Huntington's disease gene itself.
	www.healthline.com /search?q1=huntingtin (539 words)

	Huntingtin contains a highly conserved nuclear export signal -- Xia et al. 12 (12): 1393 -- Human Molecular Genetics (Site not responding. Last check: 2007-11-05)
		Huntingtin contains a highly conserved nuclear export signal -- Xia et al.
		that huntingtin may be dynamic between these compartments.
		Wild-type huntingtin participates in protein trafficking between the Golgi and the extracellular space
	hmg.oxfordjournals.org /cgi/content/abstract/12/12/1393 (674 words)

	Wild type huntingtin reduces the cellular toxicity of mutant huntingtin in mammalian cell models of Huntington's ...
		Wild type huntingtin reduces the cellular toxicity of mutant huntingtin in mammalian cell models of Huntington's disease -- Ho et al.
		Wild type huntingtin reduces the cellular toxicity of mutant huntingtin in mammalian cell models of Huntington's disease
		A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure
	jmg.bmjjournals.com /cgi/content/abstract/38/7/450 (527 words)

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