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
  • 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 .
  • 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).
  • HY-150236
    FITC-labeled Tominersen
    		Inhibitor
    FITC-labeled Tominersen is 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).
  • 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.
  • 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.
  • HY-132594
    Lexanersen
    Lexanersen (WVE-120102) is an antisense oligonucleotide used for the study of Huntington's disease.
Cat. No. Product Name / Synonyms Application Reactivity