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anti-Human Huntingtin Antibodies:
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Chicken Polyclonal Huntingtin Primary Antibody for IHC (fro), ELISA - ABIN538469
Lee, Yoshihara, Littleton: Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease. in Proceedings of the National Academy of Sciences of the United States of America 2004
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Human Polyclonal Huntingtin Primary Antibody for ELISA, ICC - ABIN4320700
Hall, Georgel: CHD proteins: a diverse family with strong ties. in Biochemistry and cell biology = Biochimie et biologie cellulaire 2007
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Human Monoclonal Huntingtin Primary Antibody for IHC (fro), IP - ABIN2474102
Moleman, Versluis, Bruinvels: Is morphine-induced catalepsy related to activation of dopaminergic neurones? in Psychopharmacology 1979
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Bird (Avian) Polyclonal Huntingtin Primary Antibody for IF, IHC (p) - ABIN6254174
Meunier, Merienne, Jollé, Déglon, Pellerin: Astrocytes are key but indirect contributors to the development of the symptomatology and pathophysiology of Huntington's disease. in Glia 2018
Human Monoclonal Huntingtin Primary Antibody for ELISA, WB - ABIN516379
Thompson, Aiken, Kaltenbach, Agrawal, Illes, Khoshnan, Martinez-Vincente, Arrasate, ORourke, Khashwji, Lukacsovich, Zhu, Lau, Massey, Hayden, Zeitlin, Finkbeiner, Green, LaFerla, Bates, Huang et al.: IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. ... in The Journal of cell biology 2009
Human Monoclonal Huntingtin Primary Antibody for IHC (fro), IP - ABIN2474103
Tseng, Hsu: A kinetic analysis of the repair of radiation-induced DNA double-strand breaks. in Radiation research 1990
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These investigations demonstrate a specific 'rate-limiting' role for huntingtin in formation of the telencephalon and the pre-placodal region, and differing levels of requirement for huntingtin function in specific nerve cell types.
the effects of Htt deficiency in early zebrafish development.
In vivo, huntingtin deficient zebrafish had a severe phenotype and reduced expression of LXR reg'd genes. An LXR agonist partially rescued the phenotype and expression of LXR target genes in huntingtin deficient zebrafish during early development.
Jp modifies the neuronal degeneration in a Drosophila model of Huntington's disease, and it has uncoveedr an unsuspected functional relationship with the Notch pathway.
Data strongly suggest that mutant Htt expression exclusively in all the CNS and PNS neurons leads to fluctuation of body weight, carbohydrate and protein stores, global lipid levels along with the intracellular lipid deposits through its effect on the integrated process of metabolic homeostasis. These results also support the idea that lipid metabolism remains centrally affected in HD leading to altered body weight.
These results therefore identify native Htt as a regulator of synaptic capture and neuropeptide storage.
Mutant HTT causes severe mislocalization and aggregation of nucleoporins and defective nucleocytoplasmic transport.
Early-onset sleep defects in mutated HTT Drosophila models of Huntington's disease reflect alterations of PKA/CREB signaling.
Htt aggregates cause non-cell-autonomous pathology, including loss of vulnerable neurons that can be prevented by inhibiting endocytosis in these neurons.
Glia regulate steady-state numbers of Htt aggregates expressed in neurons through a clearance mechanism that requires the glial scavenger receptor Draper and downstream phagocytic machinery.
findings support a role for HTT on dynamin 1 function and ER homoeostasis. Proteolysis-induced alteration of this function may be relevant to disease.
Htt modulated histone H3K9 methylation levels at the heterochromatin-euchromatin boundary.
In Drosophila, Huntingtin genetically interacts with autophagy pathway components.
Decreased O-linked GlcNAcylation protects from cytotoxicity mediated by huntingtin exon1 protein fragment
Loss of huntingtin protein results in the disruption of Rab11 vesicle transport.
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 regulators of mutant Htt aggregation
genes related to nuclear transport, nucleotide processes, and signaling are modifiers of huntingtin aggregation
Data indicate that Drosophila and Human Htt share biological properties, and confirm a model whereby Engrailed activates endogenous dhtt, which in turn prevents polyQ-hHtt-induced phenotypes
mutant ataxin-1 and huntingtin induce developmental and late-onset neuronal pathologies in Drosophila models
dHtt is required for maintaining the mobility and long-term survival of adult animals, and for modulating axonal terminal complexity in the adult brain.
mutant huntingtin (mHTT) and neurofilament light (NfL) protein concentrations in cerebrospinal fluid (CSF) and blood in parallel with clinical evaluation and magnetic resonance imaging in premanifest and manifest Huntington disease mutation carriers, were examined.
Salivary levels of total huntingtin are elevated in Huntington's disease patients
huntingtin SNP alters post-translational modification and pathogenic proteolysis of the protein causing Huntington disease
HTT gene region was functionally characterized through an in silico analysis using publicly available data sets. Putative regulatory regions outside of the immediate HTT promoter region have been identified with specific protein-protein interactions.
Data show that allele-selectivity in ex vivo patient cells is highly huntingtin (HTT) SNP-dependent, with implications for clinical trial target selection.
we used an amplification-free protocol for targeted enrichment utilizing the CRISPR/Cas9 system (No-Amp Targeted sequencing) in combination with single molecule, real-time (SMRT) sequencing for studying repeat elements in the huntingtin (HTT) gene, where an expanded CAG repeat is causative for Huntington disease.
HSP90 interacts with Htt-N90 on the N-terminal amphipathic alpha-helix, and then recruits USP19 to modulate the protein level and aggregation of Htt-N90.
We show that FAN1 binds to the expanded HTT CAG repeat DNA and its nuclease activity is not required for protection against CAG repeat expansion. These data shed new mechanistic insights into how the genetic modifiers of Huntington's disease (HD) act to alter disease progression and show that FAN1 affects somatic expansion of the CAG repeat through a nuclease-independent mechanism.
Huntington's disease multiple features correlate with CAG repeat length of the HTT gene.
Results found that Huntingtin-exon1 aggregates are disintegrated by VCP protein which N-terminal part (Cdc48_N domain) interacts with the N-terminal 17-amino acid region of Huntingtin-exon1.
a comprehensive analysis of the Htt interactome has been carried out and is discussed to provide a global picture of the Htt's structure-function relationships.
Study provides evidence that nucleated branching in vitro plays a critical role in the formation of complex fibrillar HTT exon-1 aggregates with multiple ends.
Data found that Httex1 (soluble Htt exon 1)with longer polyQ sequences leads to gain-of-function interactions with other proteins. Notable among the gain-of-function interactions are those with the RNA binding protein Fus and other RNA binding proteins.
our results indicate ATF7IP as a potential target to correct aberrant H3K9me3 levels induced by mutant HTT.
he structural model, validated by EPR distance measurements, illuminates the role of the htt(NT) domain in the earliest stages of prenucleation and oligomerization, before fibril formation.
folding equilibrium of huntingtin exon 1 monomer depends on its polyglutamine tract
results provide evidence for a novel function for the amphipathic helix derived from exon1 of wild-type huntingtin
Electron microscopy showed that deletion of three CAG triplets or an HTT gene knockout had no significant influence on the cell structure. The insertion of 150 CAG repeats led to substantial changes in quantitative and morphological parameters of mitochondria and increased the association of mitochondria with the smooth and rough endoplasmic reticulum while causing accumulation of small autolysosomes in the cytoplasm.
We therefore designed a longitudinal multimodal in vivo MRI, DTI and MRS study to investigate structural and neurochemical alterations associated with the disorder at different stages and brain structures (i.e. PFC, striatum, GP, hippocampus and thalamus), to understand the nature of neural changes in transgenic YAC128 mice, expressing the human full-length mutant HTT
These results suggest that Twist1 is an important upstream mediator of mutant Htt-induced neuronal death and may in part operate through epigenetic mechanisms.
the hypothesis is here raised in the sense that nuclear deposition of mutated huntingtin in adrenal chromaffin cells of R6/1 mice could be primarily responsible for poorer Na(+) channel expression
Early embryonic deletion of huntingtin from the developing pallium yields reduced numbers of cortical neurons and reduced cortical volume in adults but no evident abnormalities in cortical lamination. Striatal neurons were also reduced in their abundance, as was striatal volume, but striatal neurons were normal in their neurochemistry.
Study shows Huntington as a key mediator of fragile X mental retardation protein regulation of mitochondria unveils a previously unknown cross-talk between these two human disease genes.
We also found that NEAT1L overexpression protects from mHTT-induced cytotoxicity, while reducing it enhanced mHTT-dependent toxicity
The neurotoxicity of mHTT seeds in an inducible Drosophila model transgenic for HTTex1.
Indeed, beside other effects, A-971432 reduced the formation of mHtt aggregates in carotid arteries, which, albeit do not represent the classical brain vessels, may likely mirror brain vasculature response to the treatment
Transient knock-down of Meg3 and Neat1 in cell models of HD led to a significant decrease of aggregates formed by mutant huntingtin and downregulation of the endogenous Tp53 expression
RNA polymerase II transcription speed regulates the levels of HTTexon1 production.
Herp is a newly identified huntingtin-interacting protein that is able to reduce the cytotoxicity of mutant huntingtin by inhibiting its aggregation and promoting its degradation.
Rictor striatal levels could counteract neuronal dysfunction induced by mutant huntingtin.
This study reports that the gain-of-function mutation of full-length basally-expressing Htt in Huntington's disease cell Q111 (STHdhQ111/HdhQ111) upregulated microRNA-214 and decreased beta catenin & its transcriptional activity in an aggregate-independent manner.
This study conclude that expanded polyglutamine repeats of htt protein influence Huntington's Disease pathogenesis in a sex-dependent manner
expression of mutant HTT in Sim1 cells may play a role for the development of metabolic dysfunction and depressive-like behavior in male BACHD mice.
full-length endogenous Htt, which was immunoprecipitated from HD knock-in mouse and human post-mortem brain, is suitable for detection of PTMs by mass spectrometry
Knockdown of DNMT3A or DNMT1 protected neurons against mutant Htt-induced toxicity, together demonstrating a requirement for DNMTs in mutant Htt-triggered neuronal death and suggesting a neurodegenerative mechanism based on DNA methylation-mediated transcriptional repression.
Soluble mutant Httex1 stimulated the largest changes in the Neuro2a cells tanscriptome. Inactivated CREB signalling is the most profound impact arising from soluble Httex1.
This study demonstrates that loss of Htt function during neural development leads to HD-like neurological abnormalities in mice.
treating BACHD cortical neurons with ApiCCT1 prevented BACHD striatal neuronal atrophy by enhancing release of BDNF that subsequently acts through tyrosine receptor kinase B (TrkB) receptor on striatal neurons. Our findings are evidence that TRiC reagent-mediated reductions in mHTT enhanced BDNF delivery to restore the trophic status of BACHD striatal neurons.
identify 4 huntingtin-targeting miRNAs viz. miR-125b, miR-146a, miR-150 and miR-214 as candidate miRNAs responsible for observed inhibitory effect of HSF1 on huntingtin expression.
Huntingtin is a disease gene linked to Huntington's disease, a neurodegenerative disorder characterized by loss of striatal neurons. 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. A fairly broad range in the number of trinucleotide repeats has been identified in normal controls, and repeat numbers in excess of 40 have been described as pathological. 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. It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues. The larger transcript is approximately 13.7 kb and is expressed predominantly in adult and fetal brain whereas the smaller transcript of approximately 10.3 kb is more widely expressed. The genetic defect leading to Huntington's disease may not necessarily eliminate transcription, but may confer a new property on the mRNA or alter the function of the protein. One candidate is the huntingtin-associated protein-1, highly expressed in brain, which has increased affinity for huntingtin protein with expanded polyglutamine repeats. This gene contains an upstream open reading frame in the 5' UTR that inhibits expression of the huntingtin gene product through translational repression.
, Huntington's disease protein
, huntingtin (Huntington disease)
, solute carrier family 6 (neurotransmitter transporter, serotonin), member 4
, Huntington disease
, huntington disease protein
, HD protein homolog
, Huntington disease gene homolog
, huntington disease protein homolog