2. Signaling Pathways
  3. Neuronal Signaling
  4. Huntingtin

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.

Cat. No. Product Name Effect Purity Chemical Structure
  • HY-143218
    TPE-MI
    		98.54%
    TPE-MI (Tetraphenylethene maleimide) is a thiol probe for measuring unfolded protein load and proteostasis in cells. 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 parasitesPlasmodium falciparum .
    TPE-MI
  • HY-16009
    Buntanetap
    		Inhibitor 98.11%
    Buntanetap ((+)-Phenserine) is a multiple neurotoxic protein translation inhibitor with oral activity, including amyloid precursor protein (APP), α-synuclein (αSYN) and huntingtin protein (HTT). Buntanetap has anti-inflammatory effects and can be used in the study of Alzheimer's disease and Parkinson's disease.
    Buntanetap
  • HY-132579
    Tominersen
    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).
    Tominersen
  • HY-150236
    FITC-labeled Tominersen sodium
    		Inhibitor
    FITC-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).
    FITC-labeled Tominersen sodium
  • HY-132593
    Rovanersen
    Rovanersen (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.
    Rovanersen
  • HY-132594A
    Lexanersen sodium
    		Inhibitor
    Lexanersen sodium is an antisense oligonucleotide used for the study of Huntington's disease.
    Lexanersen sodium
  • HY-150245
    mHTT-IN-1
    		Inhibitor
    mHTT-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.
    mHTT-IN-1
  • HY-132594
    Lexanersen
    Lexanersen (WVE-120102) is an antisense oligonucleotide used for the study of Huntington's disease.
    Lexanersen
  • HY-156103
    mHTT-IN-2
    		Inhibitor
    mHTT-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.
    mHTT-IN-2
Cat. No. Product Name / Synonyms Application Reactivity