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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 ELISA Kits) reg'd genes. An LXR (show NR1H3 ELISA Kits) agonist partially rescued the phenotype and expression of LXR (show NR1H3 ELISA Kits) target genes in huntingtin deficient zebrafish during early development.
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 (show CREB ELISA Kits) 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 ELISA Kits) 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.
cells expressing mutant huntingtin have a dysregulated transcriptional response to epidermal growth factor (show EGF ELISA Kits) stimulation
This study demonstrated that the number of repeats in HTT, below disease threshold, confers advantageous changes in brain structure and general intelligence (IQ): the higher the number of repeats, the greater the change in brain structure, and the higher the higher IQ.
This study explores the aggregation and toxicity of human huntingtin in yeast.
Mutation in HTT causes Huntington's disease (HD); aggregates of mutated HTT cause apoptosis in neurons of HD patients. Data suggest that both MLF1 (show MLF1 ELISA Kits) and MLF2 preferentially interact with mutated N-terminal HTT; MLF1 (show MLF1 ELISA Kits)/MLF2 reduce number of neurons (Neuro2A cell line) containing mutant HTT aggregates and subsequent apoptosis. (HTT = Huntingtin protein; MLF = myeloid leukemia (show BCL11A ELISA Kits) factor)
Study investigated the fundamental intracellular aggregation process of eight different-length N-terminal fragments of Htt in both short (25Q) and long polyQ (97Q). PolyQ-expansion was only required for fragments of less than 171 amino acids to aggregate. Longer fragments aggregated predominately through a non-polyQ mechanism, involving at least one, and probably more distinct clustering mechanisms.
Study shows that mutant huntingtin protein (mHTT) has an expanded polyglutamine stretch that may adopt multiple conformations and has a slower degradation rate due to its resistance to selective autophagy in human cells and brains, revealing mechanisms of its higher toxicity.
results suggest that subject-to-subject variation contributes more to variability in N-terminal huntingtin fragments than post mortem delay
We report that endogenous huntingtin protein directly participates in oxidative DNA damage repair. Using novel chromobodies to detect endogenous human huntingtin in live cells, we show that localization of huntingtin to DNA damage sites is dependent on the kinase activity of ataxia telangiectasia mutated (ATM (show ATM ELISA Kits)) protein.
the long HTT 3'UTR (show UTS2R ELISA Kits) suppresses translation.
Soluble oligomers of PolyQ-expanded huntingtin target a multiplicity of key cellular factors.
Mutant htt is implicated in Huntington's disease-related alterations of neurotransmission.
The authors show that disease-inducing Huntingtin (mHtt) protein fragments display three distinct dynamic states in living cells - 1) fast diffusion, 2) dynamic clustering and 3) stable aggregation.
Study investigates the consequence of deleting mouse Huntingtin's (Htt's) N17 domain or a combination of its polyQ stretch and PRR (show PVRL1 ELISA Kits) (QP) on normal Htt function in mice. findings support the hypothesis that Htt's N17 and QP domains are dispensable for its critical functions during early embryonic development, but are likely more important for Htt functions in CNS development or maintenance.
Mutant Htt reduces expression of alphaB-crystallin (show CRYAB ELISA Kits) in astrocytes to decrease exosome secretion in Huntigton disease brains.
Since the R6/2 mice represent a 'truncated' huntingtin (Htt) mouse model of Huntington's disease (HD), we tested the efficacy of bezafibrate in a 'full-length' Htt mouse model, the BACHD mice. Bezafibrate treatment restored the impaired PPARg (show PPARG ELISA Kits), PPARd (show PPARD ELISA Kits), PGC (show PGC ELISA Kits)-1a signaling pathway, enhanced mitochondrial biogenesis and improved antioxidant defense in the striatum of BACHD mice
treatment of topotecan, a brain-penetrating topoisomerase 1 (show TOP1 ELISA Kits) inhibitor, to HD transgenic mouse considerably improved its motor behavioural abnormalities. Finally, we show that topotecan treatment to HD mouse not only inhibits the expression of transgenic mutant huntingtin, but also at the same time induces the expression of Ube3a (show ube3a ELISA Kits)
The present data emphasize the relevance of expanded CAG RNA to Huntington's disease pathogenesis, indicate that inhibition of HTT expression is not required to reverse motor deficits, and further suggest a therapeutic potential for LNA-CTG in polyglutamine disorders.
Study found that Q175FDN mice exhibited earlier onset and a greater variety and severity of Huntington disease (HD)-like phenotypes as compared to Q175F mice. The characterization of Q175FDN mice suggests this model offers an improved reproduction of HD phenotypes with the mutation in the knocked-in heterozygous state.
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