Huntington's Disease Related Research Tools
Huntington's disease (HD) is a lethal autosomal dominant and progressive neurodegenerative disorder, that is characterized by motor, cognitive, and behavioral impairment. At present, there are no disease-modifying treatments available other than some approaches to address certain specific symptoms of HD. HD is caused by a tandem repeat expansion mutation so that the trinucleotide (CAG) is expanded to become (CAG)n+x in the huntingtin (HTT) gene. This tandem repeat tract is transcribed and translated to become a (Q)n+x polyglutamine tract in the HTT protein. This mutant protein (mutHTT) leads to a cascade of molecular, cellular and systems changes, which, together with the modulatory actions of genetic and environmental modifiers, ultimately lead to the onset of disease symptoms.
Address mutHTT Modification and Degradation
Post-translational modifications (PTMs) of the HTT protein play an important role in the pathogenesis of HD. HTT is likely modified by SUMOylation, phosphorylation, palmitoylation, acetylation and these PTMs are important in proper protein-protein interactions of HTT. Histone acetyltransferase (HAT) enzymes CBP and PCAF were found to be inactivated by mutHTT through protein-protein interactions, leading to transcriptional and chromatin remodeling deregulation and contributing to the pathogenesis of HD. Thus, PTMs of HTT be exploited for therapeutic purposes to enhance the clearance of mutHTT. Besides, certain inhibitors of histone deacetylases (HDAC), deacetylase enzymes and mTOR have been shown to address mutHTT modification and degradation.
Address Signaling Pathways
One important HD pathology-associated change is in the cyclic AMP (cAMP) signaling and aberrant transcription of genes regulated by the cAMP response element (CRE). Inhibition of phosphodiesterase (PDE) 10A is shown to be beneficial against HD via restoration of CRE-mediated gene expression. Another important signaling pathway that contributes to the pathology of HD is MAPK signaling.
Fig.1 Potential pathological molecular events in HD and possible therapeutic interventions. (Huang, 2016)
Decrease mutHTT Content
Reducing the content of mutHTT by inhibiting gene transcription, mRNA translation or promoting the breakdown of mRNA coding for HTT, may reduce any associated downstream damaging effects of mutHTT.
By using more sophisticated approaches incorporating modelling, computational biology and systems neuroscience, Creative Biolabs can obtain a more comprehensive and integrated understanding of the pathogenesis of HD, which will have major implications for therapeutic approaches aimed at preventing, treating and ultimately curing this devastating disease. Creative Biolabs offers comprehensive products for you. Products range from neural cell lines, medium, cell dyes to customized neural antibodies, peptides, recombinant proteins and assay kits.
Targets Related to HD
Process | Strategy | Popular Targets |
Address mutHTT Modification and Degradation |
HDAC Inhibitor Deacetylase Enzymes Inhibitors mOTR Inhibitor |
HDAC Deacetylase Enzymes mOTR |
Address Signaling Pathways |
PDE10A Inhibitor; MKP-1 inhibitor; MLK-2 Inhibitor; Kynurenine Monooxygenase (KMO) Inhibitors; Extracellular Signal-regulated Kinase (ERK) Activator; Transcription Factors Activator; cmGluR5 Antagonist |
PDE 10A; MKP-1; MLK-2; KMO; ERK1/ERK 2; CREB; Elk-1; GPCR; mGluR5; PGC-1α |
Decrease mutHTT Content |
Transcription by zinc finger proteins (ZFPs) Inhibitors mutHTT Targeting Antisense Oligonucleotides (ASOs) |
ZFP; mutHTT mRNA; mutHTT Protein |
Reference
- Huang, W. J.; et al. Huntington's disease: Molecular basis of pathology and status of current therapeutic approaches. Experimental and therapeutic medicine. 2016, 12(4): 1951-1956.
Target
Anti-HA-Tag Monoclonal Antibody
- Host Species:
- Mouse
- Applications:
- IF; IP; WB; ELISA; IHC
- Conjugation:
- Unconjugated
- NeuroMab™ Mouse Anti-EFNB2 Monoclonal Antibody (CBP1159) (Cat#: NAB-0720-Z4396)
- NeuroMab™ Anti-GARP Antibody(NRP-0422-P1639) (Cat#: NRP-0422-P1639)
- Tau Monoclonal Antibody (AT120, HT7 and BT2 clone) (Cat#: NK-2106-P008)
- NeuroMab™ Anti-ApoC3 BBB Shuttle Antibody(NRZP-1022-ZP3503) (Cat#: NRZP-1022-ZP3503)
- NeuroMab™ Anti-Integrin αvβ8 BBB Shuttle Antibody(NRZP-1222-ZP1218) (Cat#: NRZP-1222-ZP1218)
- NeuroMab™ Anti-GD2 Antibody(NRZP-1222-ZP767) (Cat#: NRZP-1222-ZP767)
- NeuroMab™ Mouse Anti-LRP1 Monoclonal Antibody (CBP3363) (Cat#: NAB-0720-Z6479)
- NeuroMab™ Anti-PVRIG Antibody(NRP-0422-P792) (Cat#: NRP-0422-P792)
- NeuroMab™ Anti-F-Spondin/SPON1 Antibody, Clone N24875P (CBP11839) (Cat#: NRZP-0822-ZP4740)
- NeuroMab™ Rabbit Anti-LRRK2 Monoclonal Antibody (CBP1887) (Cat#: NAB-08-PZ735)
- Human CMEC/D3 Cell Line, Blood-Brain Barrier Model (Cat#: NCL-2108-P020)
- Mouse Hippocampal Neuron Cell HT22 (Cat#: NCL2110P001)
- Mouse Retinal Ganglion Cell Line RGC-5 (Cat#: NCL2110P154)
- Human Hippocampal Neuron Cells HPPNCs (Cat#: NCL2110P106)
- Rat Schwann Cells RSC96, Immortalized (Cat#: NCL-2108P21)
- iNeu™ Retinal Pigment Epithelial Cells (RPE) (Cat#: NRZP-0323-ZP92)
- Green Fluorescent Alpha-synuclein SH-SY5Y Cell Line (Cat#: NCL2110P209)
- Human Brain Vascular Pericytes (Cat#: NCL-21P6-015)
- C57 Brain Cortex Neurons [Mouse] (Cat#: NCC20-9PZ48)
- Mouse Retinal Ganglion Cells (Cat#: NCL2110P145)
- Human Tau Aggregation Kit (Cat#: NRP-0322-P2173)
- Beta Amyloid (1-42), Aggregation Kit (Cat#: NRZP-0323-ZP200)
- Alpha-Synuclein Aggregation Assay Kit (Cat#: NRZP-1122-ZP37)
- Alpha Synuclein Aggregation Kit (Cat#: NRZP-1122-ZP15)
- Amyloid beta 1-42 Kit (Cat#: NRP-0322-P2170)
- Beta Amyloid (1-40), Aggregation Kit, TTF Assay (Cat#: NRZP-0323-ZP199)
- Human Poly ADP ribose polymerase,PARP Assay Kit (Cat#: NRZP-1122-ZP62)
- Human GFAP ELISA Kit [Colorimetric] (Cat#: NPP2011ZP383)
- AAV2/9-hEF1a-fDIO-eNpHR 3.0-mCherry-WPRE-pA (Cat#: NTA-2012-ZP78)
- pAAV-syn-jGCaMP8s-WPRE (Cat#: NTA-2106-P063)
- AAV2/9-hSyn-Flpo-EGFP-WPRE-pA (Cat#: NTA-2012-ZP149)
- AAV2/9-hEF1a-DIO-mCherry-P2A-TetTox-WPRE-pA (Cat#: NTA-2012-ZP268)
- pAAV-syn-jGCaMP8f-WPRE (Cat#: NTA-2106-P061)
- pAAV-syn-jGCaMP8m-WPRE (Cat#: NTA-2106-P062)
- rAAV-CAG-DIO-G-Flamp1 (Cat#: NRZP-0722-ZP719)
- pAAV-syn-FLEX-jGCaMP8f-WPRE (Cat#: NTA-2106-P064)
- AAV-mDLX-CRE-tdTomato (Cat#: NRZP-0622-ZP721)
- AAV2/2Retro-CAG-DIO-EGFP-2A-TetTox-pA [Neural Tracing] (Cat#: NTA-2012-ZP303)
- Lenti of Mouse synuclein, alpha (Snca) transcript variant (NM_001042451) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0864)
- Rat Parkinson disease (autosomal recessive, juvenile) 2, parkin (Park2) (NM_020093) ORF clone/lentiviral particle, Myc-DDK Tagged (Cat#: NEP-0621-R0041)
- App Rat amyloid beta (A4) precursor protein (App)(NM_019288) ORF clone, Untagged (Cat#: NEP-0421-R0053)
- Lenti of Human TAR DNA binding protein (TARDBP) (NM_007375) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0832)
- Human huntingtin-associated protein 1 (HAP1) transcript variant 2 (NM_177977) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0676)
- ABCA1 Antisense Oligonucleotide (NV-2106-P27) (Cat#: NV-2106-P27)
- Human apolipoprotein E (APOE) (NM_000041) ORF clone, Untagged (Cat#: NEP-0421-R0232)
- Human huntingtin (HTT) (NM_002111) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0497)
- Human superoxide dismutase 3, extracellular (SOD3) (NM_003102) ORF clone, Untagged (Cat#: NEP-0521-R0808)
- Mouse Parkinson disease (autosomal recessive, early onset) 7 (Park7) (NM_020569) clone, Untagged (Cat#: NEP-0621-R0133)
- NeuroBiologics™ Rat Cerebrospinal Fluid (Cat#: NRZP-0822-ZP496)
- NeuroBiologics™ Human Cerebrospinal Fluid (Cat#: NRZP-0822-ZP491)
- NeuroBiologics™ Pig Cerebrospinal Fluid (Cat#: NRZP-0822-ZP498)
- NeuroBiologics™ Monkey Cerebrospinal Fluid (Cat#: NRZP-0822-ZP495)
- NeuroBiologics™ Mouse Cerebrospinal Fluid (Cat#: NRZP-0822-ZP497)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP509)
- NeuroPro™ Anti-EPO BBB Shuttle Protein (Cat#: NRZP-0423-ZP508)
- NeuroPro™ Anti-IDS BBB Shuttle Protein (Cat#: NRZP-0423-ZP503)
- NeuroPro™ Anti-Erythropoietin BBB Shuttle Protein (Cat#: NRZP-0423-ZP499)
- NeuroPro™ Anti-ASA BBB Shuttle Protein (Cat#: NRZP-0423-ZP504)
- NeuroPro™ Anti-NAGLU BBB Shuttle Protein (Cat#: NRZP-0423-ZP506)
- NeuroPro™ Anti-PON1 BBB Shuttle Protein (Cat#: NRZP-0423-ZP507)
- NeuroPro™ Anti-TNFR BBB Shuttle Protein (Cat#: NRZP-0423-ZP510)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP500)
- NeuroPro™ Anti-IDUA BBB Shuttle Protein (Cat#: NRZP-0423-ZP502)