Huntingtin
Huntingtin (HTT) is a soluble 3144 amino acid (348 kDa) protein, with the highest levels of expression being found in the CNS and testes. An abnormal expansion of a glutamine stretch (polyQ) in N-terminal sequence of huntingtin leads to the devastating neurodegenerative disorder Huntington's disease (HD). Subcellular fractionation and microscopic studies have shown that huntingtin is associated with vesicles and microtubules by interacting with huntingtin-associated protein 1 (HAP1), which is reported to form a complex with the dynactin and modulate or regulate the dynein–dynactin complex. There is evidence that huntingtin participates in post-Golgi trafficking of proteins that follow the regulated secretory pathway.
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Huntingtin Related Products (29)
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Antibodies (3)
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TPE-MI
0 ImagesSynonyms: Tetraphenylethene maleimideTPE-MI (Tetraphenylethene maleimide) is a thiol probe for measuring unfolded protein load and proteostasis in cells (the excitation wavelength is 350 nm and the emission wavelength is 470 nm). TPE-MI can report imbalances in proteostasis in induced pluripotent stem cell models of Huntington disease, as well as cells transfected with mutant Huntington exon 1 before the formation of visible aggregates. TPE-MI also detects protein damage following dihydroartemisinin research of the malaria parasites Plasmodium falciparum . -
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Votoplam
0 ImagesSynonyms: PTC518Votoplam (PTC518) (Example 37) is an HTT gene regulator with an IC50 ≤ 0.1 μM. Votoplam can be used in the research of Huntington's disease. -
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Desonide
0 ImagesDesonide is a non-fluorinated corticosteroid anti-inflammatory agent that acts on the glucocorticoid receptor. Desonide can also specifically bind to the mutant huntingtin protein (mHTT), reducing the level and toxicity of mHTT. Desonide can be used in the research of Huntington's disease and inflammatory diseases such as atopic dermatitis. -
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Tominersen sodium
0 ImagesCat. No.: HY-132579ASynonyms: RG6042 sodium; IONIS-HTTRx sodiumTominersen sodium is a second-generation 2′-O-(2-methoxyethyl) antisense oligonucleotide that targets huntingtin protein (HTT) mRNA and potently suppresses HTT production. Tominersen improves survival and reduces brain atrophy in mice. Tominersen sodium can be used for the research of Huntington’s disease (HD). -
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SEN177
0 ImagesSEN177 is an orally effect glutamine cyclase (QC) inhibitor. The Ki of SEN177 for human glutamine cyclase (hQC) is 20 nM, and the IC50 is 13 nM. SEN177 interferes with the interaction between CD47 and SIRRPα, and has anti-tumor activity. SEN177 reduces aggregation and apoptosis caused by HTT mutation in Huntington model, and can be used in the study of neurodegenerative diseases. -
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Brezosiran
0 ImagesCat. No.: HY-185316CAS No.: 3059403-91-4Brezosiran is a siRNA that inhibits huntingtin synthesis. -
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Brezosiran sodium
0 ImagesCat. No.: HY-185316ABrezosiran sodium is a siRNA that inhibits huntingtin synthesis. -
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PROTAC mHTT Degrader-1
0 ImagesCat. No.: HY-181879CAS No.: 2919475-43-5PROTAC mHTT Degrader-1 is a blood-brain barrier-penetrant mutant huntingtin (mHTT) PROTAC degrader. PROTAC mHTT Degrader-1 specifically recognizes pathogenic mHTT aggregates and recruits Cereblon (CRBN), thereby inducing ubiquitination and proteasomal degradation of mHTT. PROTAC mHTT Degrader-1 alleviates mHTT-induced cytotoxicity and neuroinflammation. In the R6/2 Huntington's disease mouse model, PROTAC mHTT Degrader-1 reduces cerebral protein aggregation levels and improves body weight, motor coordination and survival rate of animals. PROTAC mHTT Degrader-1 can be used for research on PROTAC therapies for Huntington's disease and other neurodegenerative diseases. -
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mHTT-IN-2
0 ImagesmHTT-IN-2 (compound 27) is a potent inhibitor (EC50=0.066 μM) of mutant huntingtin (mHTT). mHTT-IN-2 reduces canonical splicing of HTT RNA exons [49-50] and is a splicing regulator of the huntingtin (HTT) gene. mHTT-IN-2 exhibits inhibitory activity in vitro and in vivo in human HD stem cells and mouse BACHD models. mHTT-IN-2 may be used in the study of branaplam-related peripheral neuropathy. -
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Tominersen
0 ImagesCat. No.: HY-132579CAS No.: 1709886-74-7Synonyms: RG6042; IONIS-HTTRxTominersen (RG6042) is a second-generation 2′-O-(2-methoxyethyl) antisense oligonucleotide that targets huntingtin protein (HTT) mRNA and potently suppresses HTT production. Tominersen improves survival and reduces brain atrophy in mice. Tominersen can be used for the research of Huntington’s disease (HD). -
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Dehydrothio-p-toluidine
0 ImagesSynonyms: DHPTDehydrothio-p-toluidine (DHPT) is a chemical intermediate used in dyestuff production. Dehydrothio-p-toluidine shows inhalation toxicity in acute and subchronic conditions. -
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Rovanersen
0 ImagesCat. No.: HY-132593CAS No.: 2072901-32-5Synonyms: WVE-120101Rovanersen (WVE-120101) is an antisense oligonucleotide that specifically targets mutated mRNA copies of the huntington (HTT) gene without affecting healthy mRNA of HTT gene, thereby preventing the production of faulty Huntingtin protein. Rovanersen can be used for huntington’s disease research. -
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Rovanersen sodium
0 ImagesCat. No.: HY-132593ACAS No.: 2231347-41-2Synonyms: WVE-120101 sodiumRovanersen sodium is an antisense oligonucleotide that specifically targets mutated mRNA copies of the huntington (HTT) gene without affecting healthy mRNA of HTT gene, thereby preventing the production of faulty Huntingtin protein. Rovanersen sodium can be used for huntington’s disease research. -
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PolyQ aggregation inhibitor C2-8
0 ImagesSynonyms: Polyglutamine Aggregation inhibitor IIIC2-8 is an inhibitor of polyglutamine (polyQ) aggregation (IC50s=25 and 0.05 μM for recombinant HDQ51 and in PC12 cells, respectively). It also inhibits polyQ aggregation in organotypic hippocampal slice cultures isolated from R6/2 transgenic mice and reduces neurodegeneration in a dose-dependent manner in a Drosophila model of Huntington's disease. C2-8 (100 and 200 mg/kg) reduces huntingtin aggregate size, reduces neuronal atrophy, and improves motor performance in a rotarod test in the R6/2 transgenic mouse model of Huntington's disease. -
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FITC-labeled Tominersen sodium
0 ImagesCat. No.: HY-150236FITC-labeled Tominersen (sodium) is the Tominersen labeled with FITC. Tominersen (RG6042) is a second-generation 2′-O-(2-methoxyethyl) antisense oligonucleotide that targets huntingtin protein (HTT) mRNA and potently suppresses HTT production. Tominersen improves survival and reduces brain atrophy in mice. Tominersen can be used for the research of Huntington’s disease (HD). -
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HTT-D3
0 ImagesCat. No.: HY-143792CAS No.: 2254502-89-9HTT-D3 is an orally active, blood-brain barrier penetrant splicing modulator of huntingtin (HTT). HTT-D3 promotes the inclusion of a pseudo-exon containing a premature termination codon into HTT pre-mRNA, triggers nonsense-mediated mRNA degradation and reduces HTT protein levels. HTT-D3 induces dose-dependent, comparable reductions in mutant HTT protein in both the brain and peripheral tissues of transgenic mouse models. HTT-D3 can be used for the research of Huntington's disease. -
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Polyglutamine binding peptide 1
0 ImagesPolyglutamine binding peptide 1 (QBP1) is a peptide inhibitor of polyglutamine (polyQ). Polyglutamine binding peptide 1 inhibits polyQ protein aggregation in vitro and suppresses polyQ-induced cell death in cell culture. -
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Rovanersen sodium scrambled negative control
0 ImagesCat. No.: HY-132593CRovanersen sodium scrambled negative control is the sequence scrambled negative control of Rovanersen sodium. -
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mHTT-IN-1
0 ImagesCat. No.: HY-150245CAS No.: 2839311-21-4mHTT-IN-1 (Example 1) is a potent mutant huntingtin (mHTT) inhibitor. mHTT is toxic and a major cause of the inherited autosomal dominant neurodegenerative disorder, Huntington's disease (HD). mHTT-IN-1 conducts the reduction of mHTT with an EC50 value of 46 nM. -
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Tominersen sodium scrambled negative control
0 ImagesCat. No.: HY-132579DTominersen sodium scrambled negative control is the sequence scrambled negative control of Tominersen sodium. -
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