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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 (show NR1H3 Proteins) reg'd genes. An LXR (show NR1H3 Proteins) agonist partially rescued the phenotype and expression of LXR (show NR1H3 Proteins) target genes in huntingtin deficient zebrafish during early development.
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 (show DNM1 Proteins) 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
The single overexpression of DNAJB1 (show DNAJB1 Proteins) in HEK293T cells is sufficient to profoundly reduce HttExon1Q97 aggregation and represents a target of future therapeutic avenues for HD.
The two kinases HIPK3 (show HIPK3 Proteins) and MAPK11 (show MAPK11 Proteins) effect on Huntingtin (HTT)levels are mutant HTT protein (mHTT)-dependent, providing a feedback mechanism in which mHTT enhances its own level thus contributing to mHTT accumulation and disease progression.
Lipid-based micellar nanoparticles promote aggregation of huntingtin exon-1 peptides. This study characterizes the interaction of two such peptides, htt(NT)Q7 and htt(NT)Q10 comprising the N-terminal amphiphilic domain of huntingtin followed by 7 and 10 glutamine repeats, respectively, with 8 nm lipid micelles. Monomer-dimer equilibrium exists on the surface of the micelles.
Study shows that lifetime depression risk was higher with both relatively short and relatively large Huntigtin CAG repeat (show CELF3 Proteins) sizes in the normal range.
huntingtin T3 phosphorylation is greatly reduced in samples from Huntington's disease models and in Huntington's disease patients, and T3 phosphorylation alters Huntingtin exon 1 protein conformation and aggregation properties
Multiple factors that decreased cytotoxicity of Htt-103Q (changing the length of or sequences adjacent to the polyQ, altering ploidy or chaperone dosage, or deleting anti-aging factors) altered the Htt-103Q aggregation pattern in which the suite of mid-sized aggregates at 6 hr were most correlative with cytotoxicity.
Monomeric Huntingtin Exon 1 Has Similar Overall Structural Features for Wild-Type and Pathological Polyglutamine Lengths
This study aims to establish the current state of the IT-15 (HTT) gene in different Ecuadorian ethnic groups and patients by determining CAG triplet repeats, compared with the ethnicity of individuals.
GSK3beta may inhibit VRK2 (show VRK2 Proteins) catalytic activity by disrupting its flexibility. The inhibition of VRK2 (show VRK2 Proteins) catalytic activity by GSK3beta may also inhibit VRK2 (show VRK2 Proteins)-induced degradation of TRiC (show MARVELD2 Proteins), which could suppress polyQ-expanded Htt aggregation.
cryo-electron microscopy to determine the structure of full-length human HTT in a complex with HTT-associated protein 40 (HAP40 (show F8A1 Proteins); encoded by three F8A (show F8A1 Proteins) genes in humans) to an overall resolution of 4 A
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 (show SIM1 Proteins) 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 (show DNMT3A Proteins) or DNMT1 (show DNMT1 Proteins) 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 (show HELLS Proteins)-mediated transcriptional repression.
Soluble mutant Httex1 stimulated the largest changes in the Neuro2a cells tanscriptome. Inactivated CREB (show CREB1 Proteins) 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 (show BDNF Proteins) that subsequently acts through tyrosine receptor kinase B (TrkB (show NTRK2 Proteins)) receptor on striatal neurons. Our findings are evidence that TRiC (show MARVELD2 Proteins) reagent-mediated reductions in mHTT enhanced BDNF (show BDNF Proteins) delivery to restore the trophic status of BACHD striatal neurons.
identify 4 huntingtin-targeting miRNAs viz. miR (show MLXIP Proteins)-125b, miR (show MLXIP Proteins)-146a, miR (show MLXIP Proteins)-150 and miR (show MLXIP Proteins)-214 as candidate miRNAs responsible for observed inhibitory effect of HSF1 (show HSF1 Proteins) on huntingtin expression.
cells expressing mutant huntingtin have a dysregulated transcriptional response to epidermal growth factor (show EGF Proteins) stimulation
Mutant htt is implicated in Huntington's disease-related alterations of neurotransmission.
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