huntington's disease

Tetrabenazine Metabolite ((-)-β-HTBZ), a vesicles monoamine transporter 2 (VMAT2) inhibitor, is an active metabolite of Tetrabenazine. Tetrabenazine Metabolite has a high affinity for VMAT2 with a Ki of 13.4 nM. Tetrabenazine Metabolite is often used to study chorea associated with Huntington's disease and other hyperactivity disorders.

Dalzanemdor (SAGE-718) is an NMDA receptor-positive modulator of metabolism that can be used to study Huntington's chorea, Alzheimer's disease, and cognitive dysfunction.

Meclizine, also known as Meclozine, is a piperazine class H1 antagonist with antihistamine properties that inhibits the interaction of histamine at H1 receptors reversibly. It serves as an effective anti-motion sickness agent, able to cross the blood-brain barrier. Additionally, Meclizine acts as an agonist ligand for the mouse constitutive androstane receptor (CAR) and an inverse agonist for human CAR, making it useful in research on polyQ toxicity disorders, including Huntington's disease.

BN-82451 2HCl is a dual inhibitor of COX-1 and COX-2 and can be used to study Huntington's chorea and Parkinson's disease.

1-phenyl-3,4-dihydroisochinoline (3,4-Dihydro-1-phenylisoquinoline) is a structural analog of the neurotransmitter dopamine that interacts with dopamine receptors in the central nervous system. It has been studied as a potential therapeutic agent for the treatment of neurological disorders such as Parkinson's disease, Alzheimer's disease and Huntington's chorea.

Pyrazolo[1,5-a]pyrimidine 4h is a morpholine derivative that is a selective antagonist of the adenosine A2A receptor. It has been shown to be neuroprotective in animal models of Parkinson's disease and Huntington's chorea.

HTT-D3 is a potent and orally active huntingtin (HTT) splicing modulator. HTT-D3 can lead to HTT mRNA degradation and reduction of HTT levels. HTT-D3 reduces p-glycoprotein (P-gp) efflux, and has research value in Huntington's disease.

Selisistat (EX-527) is a potent and specific inhibitor of the deacetylase SIRT1 (IC50=38 nM). Selisistat can be used in the study of neurological disorders such as Huntington's chorea.

Votoplam functions as a gene splicing modulator, employed in the inhibition of Huntington's disease [1].

MIND4-19 is an inhibitor of SIRT2 with antitumor activity.

WAY-354574 is an active compound that targets the deacetylase Sirtuin, utilized in research focused on Huntington's disease (HD) [1].

B2 (Linazolamide intermediate B impurity 2) promotes inclusion formation in cellular models of Huntington's disease and Parkinson's disease

Chloroguanabenz is an antiprion agent and a derivative of the α2-adrenergic receptor agonist guanabenz . It inhibits prion formation in yeast and mammalian cell-based assays. Chloroguanabenz reduces the levels of both soluble and aggregated forms of the truncated Huntingtin derivative Htt48 in an HEK293T cellular model of Huntington's disease.

Lexanersen (WVE-120102), an antisense oligonucleotide, is utilized in Huntington's disease research.

1-(Anilinocarbonyl)proline is a compound utilized for identifying dual-action probes within a cell model of Huntington's disease.

Rho-Kinase-IN-2 (Compound 23) is an orally active and selective inhibitor of Rho Kinase (ROCK), which can penetrate the central nervous system (CNS). It exhibits a high affinity for ROCK2 with an inhibition constant (IC50) of 3 nM. This compound is of potential interest for further investigations in the field of Huntington's disease research [1].

HDAC4 CHDI Degrader 11 is a potent, selective degrader of HDAC4 (PROTAC) with DC_50 values of 4 nM and 6 nM in Jurkat E6-1 and Jurkat cells, respectively. This compound integrates the class IIa HDAC inhibitor trifluoromethyloxadiazole with a ligand for the Von Hippel-Lindau (VHL) protein through a linker. Demonstrating significant efficacy, HDAC4 CHDI Degrader 11 achieves a DC_50 value of 1 nM in a mouse cell model of Huntington's disease. For enhanced degradation in neuroblastoma cell lines, it can be administered alongside the P-glycoprotein inhibitor, Elacridar.

SC 51089 is a selective prostaglandin receptor PGE2 antagonist with selectivity for prostaglandin receptor subtypes and antinociceptive activity that improves motor deficits and rescues memory decline in the Huntington's disease R6/1 mouse model .

Remacemide is a low-affinity NMDA antagonist with sodium channel blocking properties. It has been studied for the treatment of acute ischemic stroke, epilepsy, Huntington's disease, and Parkinson's disease.

SMER10 is an enhancer of the clearance of mutant aggregate-prone proteins by autophagy in mammalian cell models of Huntington's and Parkinson's disease, independent of rapamycin.

C2-8 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.

NSC66098 is a novel potent inhibitor of Huntington's disease (HD) pathogenesis via targeting CAG repeats RNA and Poly Q protein.

Pridopidine Hydrochloride is a dopaminergic stabilizer that has been shown to improve a variety of neurological and psychiatric disorders, including Huntington's disease. Pridopidine Hydrochloride stabilizes psychomotor activity in a state-dependent way t

MK-28 is a selective PERK agonist that reduces toxicity and prolongs survival in a Huntington's disease model.MK-28 has a significant killing effect on MK28 gastric cancer cells.

Tau Protein Aggregation-IN-1 (Compound 0c) serves as an inhibitor of Tau protein aggregation, applicable in research related to protein folding disorders including Alzheimer's disease, dementia, Parkinson's disease, Huntington's disease, and prion-based spongiform encephalopathies [1].

PF-02545920, a PDE10 inhibitor, is used potentially for the treatment of Huntington's Disease.

Dantrolene is a postsynaptic muscle relaxant by inhibiting Ca2+ ions release from sarcoplasmic reticulum stores by antagonizing ryanodine receptors.

PBT-1033 hydrochloride is a potential neuroprotective agent for the treatment of Alzheimer's disease and Huntington's disease.

Kynurenic acid, an active metabolite of tryptophan, is synthesized through a kynurenine intermediate by kynurenine aminotransferases (KATs). It acts as an antagonist of both NMDA and AMPA receptors, as well as α7 nicotinic acetylcholine receptors (nAChRs; EC50s = 235, 101, and 7 µM, respectively), and functions as an agonist for the aryl hydrocarbon receptor (AhR) and G protein-coupled receptor 35 (GPR35; EC50s = 1.4 and 39 µM, respectively). In a neonatal rat model of cerebral hypoxic-ischemia, induced by carotid artery ligation, administration of kynurenic acid at 300 mg/kg prevents weight loss in the lesioned hemisphere. Additionally, at concentrations of 1 and 5 mg/ml, it protects against neurodegeneration in the rhabdomere of the eye in an Htt93QtransgenicDrosophila model of Huntington's disease. Elevated levels of kynurenic acid in the cerebrospinal fluid have been observed in patients with schizophrenia.

PBT-1033 (PBT-2) is a potential neuroprotective agent for the treatment of Alzheimer's disease and Huntington's disease.

mHTT-IN-1 (Example 1) is a potent inhibitor of the mutant huntingtin protein (mHTT), which is toxic and primarily responsible for Huntington's disease (HD), an inherited autosomal dominant neurodegenerative disorder. This compound effectively reduces mHTT levels with an EC50 of 46 nM.

AUTEN-67 is a specific inhibitor of MTMR14 and autophagy enhancer with potent antiaging and neuroprotective effects. AUTEN-67 also delays the onset and decreases the severity of Huntington's disease.

NCT-504, a selective allosteric inhibitor of PIP4Kγ, exhibits an IC50 value of 15.8 μM. It holds promise for the investigation of Huntington's disease.