2. Disease Areas
  3. Neurological, Eye or Ear Disease
  4. Neurodegenerative Disease
  5. Huntington's Disease

Huntington's Disease

Huntington's Disease is a rare, inherited neurodegenerative disorder caused by a genetic mutation that leads to progressive degeneration of nerve cells in the brain, particularly in the basal ganglia and cerebral cortex. It typically manifests in mid-adulthood with symptoms including uncontrolled choreic movements, cognitive decline, emotional disturbances such as depression and irritability, and behavioral changes. The disease progresses through early, moderate, and late stages, eventually resulting in severe motor dysfunction, dementia, and loss of independence. A small percentage of cases present in childhood or adolescence, often with rigid akinetic symptoms rather than chorea. Huntington’s disease-like syndromes (HDLs), including HDL1, HDL2, HDL3, and HDL4 (SCA17), share clinical features with HD but differ in age of onset, inheritance patterns, and specific symptoms, with HDL3 presenting earlier and including seizures. All forms are autosomal dominant, linked to defective genes, and lead to similar neurological deterioration over time, with life expectancy after symptom onset ranging from 10 to 20 years.

Huntington's Disease (31):

Cat. No. Product Name CAS No. Purity Chemical Structure
  • HY-142035
    N-Propargylglycine 58160-95-5 99.70%
    N-Propargylglycine is a brain-penetrant and orally active PRODH inhibitor. N-Propargylglycine covalently modifies enzyme-bound FAD and active site lysine, causing enzyme structural distortion, protein decay, and irreversible inhibition of proline and 4-hydroxyproline catabolism. N-Propargylglycine induces UPRmt, upregulates mitochondrial chaperones and YME1L1, enhances mitochondrial proteostasis, blocks astrocytic L-proline consumption, and abolishes L-proline’s ATP-maintaining and viability-protective effects. N-Propargylglycine stimulates neural processes, increases brain proline, hydroxyproline, and sarcosine levels, partially normalizes Huntington’s disease whole brain transcriptomes. N-Propargylglycine reduces hyperoxaluria, prevents calcium oxalate stone formation, reduces kidney tubular damage, and restores weight and survival in Grhpr knockout mice. N-Propargylglycine can be used for the research of breast cancer, neurodegenerative disorders, Huntington’s disease, and primary hyperoxaluria type 2.
    N-Propargylglycine
  • HY-128700
    Nicotinic acid mononucleotide 321-02-8 98.08%
    Nicotinic acid mononucleotide acts as a SARM1 inhibitor and a NAD+ biosynthesis intermediate, with an IC50 value of 93.3 μM against SARM1. Nicotinic acid mononucleotide exerts axon-protective effects, delays axonal degeneration, elevates NAD+ levels, enhances Sirt1 activity, improves myocardial capillary density and alleviates myocardial fibrosis. Nicotinic acid mononucleotide reverses diabetic cardiomyopathy in diabetic mice by increasing myocardial NAD+ levels. Nicotinic acid mononucleotide is applicable to research related to cancer, multiple sclerosis, diabetic cardiomyopathy, neurodegenerative diseases and Huntington's disease.
    Nicotinic acid mononucleotide
  • HY-B0696
    Tiagabine 115103-54-3 98.49%
    Tiagabine (NO050328; NO328; TGB) is an orally active, highly selective, and reversible GAT-1 inhibitor and anticonvulsant that crosses the blood-brain barrier. By blocking the reuptake of GABA in neurons and glial cells, tiagabine increases extracellular GABA levels to enhance inhibitory signal transduction, thereby exerting multiple activities such as anticonvulsant, neuroprotective, and antioxidant effects. Tiagabine exhibits linear pharmacokinetic properties. Although it is metabolized by CYP3A and has a high protein binding rate, it carries a low risk of cognitive impairment. Tiagabine is widely used in research on related diseases including epilepsy (including refractory partial seizures), alcohol withdrawal symptoms, and Huntington's disease.
    Tiagabine
  • HY-B0590S
    Tetrabenazine-d6 1392826-25-3 ≥99.0%
    Tetrabenazine-d6 (Deutetrabenazine) is a deuterium-labled Tetrabenazine (HY-B0590). Tetrabenazine (Ro 1-9569) is a brain-penetrant and orally active VMAT2-selective ligand with human VMAT2 Ki 100 nM. Tetrabenazine binds VMAT2 to block monoamine uptake into synaptic vesicles, potentiates cytoplasmic monoamine degradation. Tetrabenazine weakly blocks dopamine D2 receptors, and increases dopamine turnover via elevated cerebrospinal fluid homovanillic acid. Tetrabenazine can be used for the research of Huntington’s disease, tardive dyskinesia, and Tourette’s syndrome.
    Tetrabenazine-d<sub>6</sub>
  • HY-103333
    Arvanil 128007-31-8 99.1%
    Arvanil (N-Vanillylarachidonamide) is a mixed agonist of CB1 and TRPV1 receptors. Arvanil downregulates CD25, HLA-DR, CD134/OX40, blocks G1/S phase transition, and induces phosphorylation of Akt. Arvanil does not induce apoptosis in cells. Arvanil inhibits lymphocyte activation and ameliorates autoimmune encephalomyelitis. Arvanil can be used in research related to Huntington's disease, vomiting, and multiple sclerosis.
    Arvanil
  • HY-186130
    CAST-calpain-2 stabilizer-1 3110929-05-7
    CAST-calpain-2 stabilizer-1 is a blood-brain barrier permeable stabilizer of CAST-calpain-2 interaction. CAST-calpain-2 stabilizer-1 shows no activity against GSK3. CAST-calpain-2 stabilizer-1 can be used in the research of Huntington's disease and tauopathies.
    CAST-calpain-2 stabilizer-1
  • HY-P6437A
    Drp1 peptide inhibitor P110 TFA 99.11%
    Drp1 peptide inhibitor P110 (Compound P110) TFA is a selective Drp1 peptide inhibitor with neuroprotective properties. Drp1 peptide inhibitor P110 TFA can inhibit the activation of Drp1, prevent MPTP (HY-15608)-induced Drp1 mitochondrial translocation, and alleviate MPTP-induced dopaminergic neuron loss, dopaminergic nerve terminal damage, and behavioral deficits, and can be used in the study of Alzheimer's disease. Additionally, Drp1 peptide inhibitor P110 TFA can reduce mitochondrial damage and organ injury in animal models of Huntington's disease, cerebral ischemic injury, and myocardial infarction.
    Drp1 peptide inhibitor P110 TFA
  • HY-181988
    DTB-acid
    DTB-acid is a multi-target inhibitor that binds stably to Caspase-6, KMO and GSK-3β. DTB-acid is applicable to the research of Huntington's disease.
    DTB-acid
  • HY-100384
    NKL 22 537034-15-4
    NKL 22 is a potent and selective inhibitor of histone deacetylases (HDAC), with IC50 values of 199 and 69 nM for HDAC1 and HDAC3, respectively. NKL 22 can reverse abnormal expression of HD‑related genes and restore the levels of key genes including Ppp1r1b in Huntington's disease transgenic mice. NKL 22 can be used for the researches of Huntington's disease and cancer.
    NKL 22
  • HY-D0193A
    Ponceau 4R (85%) 2611-82-7
    Ponceau 4R (85%) (Acid Red 18 (85%); New Coccine (85%)) is an orally active synthetic food colorant and a HSA-binding aggregator. Ponceau 4R (85%) binds to HSA, inducing its partial unfolding, conformational changes and aggregation. Ponceau 4R (85%) serves as a food colorant and can be used in research on diseases including type Ⅱ diabetes, Parkinson's disease, Huntington's disease and spongiform encephalopathy.
    Ponceau 4R (85%)
  • HY-131885
    RuBi-Glutamate hexafluorophosphate sodium 2417096-44-5
    RuBi-Glutamate hexafluorophosphate sodium is a neuronal activator. RuBi-Glutamate hexafluorophosphate sodium photocleaves to release glutamate upon one- or two-photon excitation, activating glutamate receptors in cortical pyramidal neurons. RuBi-Glutamate hexafluorophosphate sodium reduces peak amplitude of evoked GABAergic inhibitory postsynaptic currents in its caged form. RuBi-Glutamate hexafluorophosphate sodium can be used for the research of Huntington's disease.
    RuBi-Glutamate hexafluorophosphate sodium
  • HY-128128
    ASN04421891 570365-12-7 99.00%
    ASN04421891 is a GPR17 agonist with nanomolar EC50 and high specificity. ASN04421891 promotes oligodendrocyte precursor cell maturation to mature myelinating oligodendrocytes. ASN04421891 can be used for the research of cerebral ischaemia, cardiac ischaemia, renal ischaemia, cerebral trauma, multiple sclerosis, schizophrenia, depression, alzheimer's disease, alzheimer-like dementia, parkinson's disease, huntington's chorea, amyotrophic lateral sclerosis (ALS), neuroinflammatory disorders.
    ASN04421891
  • HY-143792
    HTT-D3 2254502-89-9
    HTT-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.
    HTT-D3
  • HY-156600A
    Bevemipretide trihydrochloride 2589640-11-7
    Bevemipretide trihydrochloride (SBT-272 trihydrochloride) is a blood-brain barrier-permeable mitochondrial function repair agent. Bevemipretide trihydrochloride stabilizes cardiolipin in the inner mitochondrial membrane, restores mitochondrial structure, respiratory function, motor capacity and upper motor neuron health. Bevemipretide trihydrochloride reduces astrogliosis and microgliosis in mice with amyotrophic lateral sclerosis. Bevemipretide trihydrochloride prevents stroke-induced mitochondrial dysfunction. Bevemipretide trihydrochloride is applicable to research related to amyotrophic lateral sclerosis, Parkinson's disease, dementia with Lewy bodies and Huntington's disease.
    Bevemipretide trihydrochloride
  • HY-136878
    LSD1-IN-10 1235864-15-9
    LSD1-IN-10 is a LSD1/MAO inhibitor with LSD1 IC50 of 5 nM, MAO-A IC50 of 16 μM, and MAO-B IC50 of 7.4 μM. LSD1-IN-10 suppresses enzymatic activity of LSD1, MAO-A, and MAO-B. LSD1-IN-10 can be used for the research of cancer, alzheimer's disease, parkinson's disease, huntington's disease.
    LSD1-IN-10
  • HY-182456
    CHDI-340246 1426319-74-5
    CHDI-340246 is an orally active kynurenine monooxygenase (KMO) inhibitor. CHDI-340246 blocks KMO activity, alters the metabolic flux of the kynurenine pathway, inhibits the production of 3-hydroxykynurenine and quinolinic acid, elevates the levels of kynurenine and kynurenic acid, and restores electrophysiological abnormalities in transgenic mouse models of Huntington's disease. CHDI-340246 can be used in studies related to Huntington's disease.
    CHDI-340246
  • HY-186116A
    LSD1-IN-49 hydrochloride 1422534-06-2
    LSD1-IN-49 hydrochloride (Compound (±) 1) is an irreversible LSD1/KDM1A inhibitor with an IC50 of 29 nM against hLSD1. LSD1-IN-49 hydrochloride irreversibly inhibits the enzymatic activity of LSD1 by forming an adduct with flavin adenine dinucleotide (FAD) in the binding pocket of LSD1. LSD1-IN-49 hydrochloride is applicable to research related to schizophrenia, autism spectrum disorder and Huntington's disease.
    LSD1-IN-49 hydrochloride
  • HY-181897
    mHTT ligand-1 1903786-86-6
    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-181879
    PROTAC mHTT Degrader-1 2919475-43-5
    PROTAC 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.
    PROTAC mHTT Degrader-1
  • HY-181406
    eIF2B activator-1 3118633-46-5
    eIF2B activator-1 (Compound 7a) is a eIF2B activator with a pEC50 value of 7.3. eIF2B activator-1 exhibits significant hERG inhibitory activity, with a pIC50 value of 5.7. eIF2B activator-1 can be used in research related to Huntington's disease, Parkinson's disease, and amyotrophic lateral sclerosis (ALS).
    eIF2B activator-1