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-156650A
    Votoplam hydrochloride
    		Modulator
    Votoplam (PTC518) hydrochloride is an HTT gene regulator with an IC50 ≤ 0.1 μM. Votoplam hydrochloride can be used in the research of Huntington's disease.
    Votoplam hydrochloride
  • HY-180346
    Hepta-histidine
    		Inhibitor
    Hepta-histidine is an inhibitor of Ku70-Huntingtin protein interaction. Hepta-histidine can reverse the morphological abnormalities of primary neurons differentiatied from hiPSCs. Hepta-histidine prolongs the lifespan in severe Huntington’s disease R6/2 mouse model. Hepta-histidine ameliorates DNA damage in vitro. Hepta-histidine can be used to study anti-aggregation agent against Tau-associated neurodegenerative diseases such as Alzheimer’s disease and Huntington’s disease.
    Hepta-histidine
  • HY-DY1024
    TPE-MI (solution)
    TPE-MI (Tetraphenylethene maleimide) (solution) 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 parasitesPlasmodium falciparum .
    Solvent and concentration: DMSO: 10 mM
    TPE-MI (solution)
  • HY-165539
    SMER10
    		Inhibitor
    SMER10 is a small-molecule enhancer that can induce autophagy. SMER10 can increase the number of EGFP-LC3 positive autophagosoms in COS-7 and HeLa cells, promoting the conversion of LC3-I to autophagosome-associated LC3-II. SMER10 can efficiently promote the degradation of autophagy substrates, including the mutant huntingtin protein (EGFP-HDQ74) associated with Huntington's disease and the A53T α-synuclein protein associated with Parkinson's disease. SMER10 exerts neuroprotective effect.
    SMER10
  • HY-181897
    mHTT ligand-1
    		Ligand
    mHTT ligand-1 is an mHTT ligand. As a Ligand for Target Protein for PROTAC, mHTT ligand-1 can be used to develop and design PROTAC-based mHTT degraders, such as PROTAC mHTT Degrader-1 (HY-181879). mHTT ligand-1 is applicable to research related to Huntington's disease.
    mHTT ligand-1
  • HY-B0248R
    Desonide (Standard)
    		Inhibitor
    Desonide (Standard) is the analytical standard of Desonide. This product is intended for research and analytical applications. Desonide 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.
    Desonide (Standard)
Cat. No. Product Name / Synonyms Application Reactivity