Prion Disease Related Research Tools
Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of fatal neurodegenerative disorders that include Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, kuru and fatal familial insomnia. Prion diseases are thought to be caused by the misfolding of native cellular prion protein (PrPC) into the aggregation prone form (PrPSc). PrPSc refers to the misfolded infectious form that differs from PrPC because it has a higher content of β-sheet structure and it aggregates to form medium and large size polymers. The conversion of PrPC into PrPSc is the main event in prion disease.
Fig.1 Structure of PrPC and PrPSc.
The Central Role of PrPSc
There is no doubt that the formation and accumulation of PrPSc in the brain is the triggering factor of prion disease. PrPSc accumulates in different brain regions as distinct types of deposits depending on the animal species and strains of the agent. The loss of the critical biological function of PrPC is one possible mechanism by which PrPSc formation might result in neurodegeneration. Studies have proposed various functions of PrPC, such as the roles in apoptosis, oxidative stress, neuroprotection, transmembrane signaling, myelination, cell adhesion, and trafficking of metal ions. Another possible mechanism by which PrPSc formation might be linked to the disease is by direct toxicity of the misfolded protein.
Therapeutic Development
Currently, no therapeutic or prophylactic regimens exist for prion diseases. A variety of therapeutic strategies have been proposed but most of them directed at preventing prion conversion. The targets include inhibition of PrPC expression, enhancement of PrPC degradation, inhibition of PrPC-PrPSc interaction, inhibition of PrPSc oligomer formation, and enhancement of PrPSc degradation. PrPC and PrPSc have been widely studied to understand the enigmatic nature of prions. Additionally, some cellular factors have been implicated as possible targets in PrPC-mediated or PrPSc-induced neurodegenerative processes. One example is the growth arrest and DNA-damage-inducible protein GADD34. GADD34 promotes dephosphorylation of the α-subunit of eukaryotic translation initiation factor 2 and reverses translational suppression caused by the prion infection-induced unfolded protein response in the endoplasmic reticulum.
Fig.1 Therapeutic targets in PrPSc degradation pathways. (Goold, 2015)
Targets and Canditaes Related to Prion Disease
Beclin VPS34 Complex | P150; Vps34; Beclin 1; mAtg14 |
mTROC complex | Raptor; PRAS40; mTOR; Deptor; mLST8 |
Others | Sirt 1; Heat shock proteins; Cytosolic PrPSc; Membrane associated PrPSc; Lysomal proteases; USP 14; p38 MAPK; DNA-damage-inducible protein GADD34 |
Small molecules | Prion receptor laminin (LPR/LR); Pentosan polysulfate (PPS); Quinacrine; Compound B; IND series; Anle138b; Amphotericin B |
Creative Biolabs aims to provide biological products to help promote your prion diseases project research. These reagents include but are not limited to site-specific modified peptides, conjugated peptides, monoclonal antibodies (mAbs), polyclonal antibodies (pAbs), labeled antibodies, proteins, analytical kits, and small molecule activators and inhibitors.
Reference
- Goold, R.; et al. Prion degradation pathways: potential for therapeutic intervention. Molecular and Cellular Neuroscience. 2015, 66: 12-20.
Target
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Rabbit Monoclonal Antibody to Ubiquitin
- Host Species:
- Rabbit
- Species Reactivity:
- Human
- Applications:
- Sandwich ELISA; WB; IP; FC; IHC-P; ICC; IF; ELISA
- Conjugation:
- Unconjugated; APC; PE; HRP; Biotin; FITC; Alexa Fluor 488; Alexa Fluor 700; Alexa Fluor 647; Alexa Fluor 750; Alexa Fluor 594; Alexa Fluor 350; Alexa Fluor 1453
- NeuroMab™ Anti-Tau Antibody(NRP-0422-P2293) (Cat#: NRP-0422-P2293)
- NeuroMab™ Anti-PVRIG Antibody(NRZP-1022-ZP4114) (Cat#: NRZP-1022-ZP4114)
- NeuroMab™ Anti-Tau Antibody(NRP-0422-P1684) (Cat#: NRP-0422-P1684)
- NeuroMab™ Mouse Anti-SHANK3 Monoclonal Antibody (CBP929) (Cat#: NAB-0720-Z3477)
- NeuroMab™ Anti-PVRIG Antibody(NRP-0422-P515) (Cat#: NRP-0422-P515)
- NeuroMab™ Anti-ApoC3 BBB Shuttle Antibody(NRZP-1022-ZP3503) (Cat#: NRZP-1022-ZP3503)
- NeuroMab™ Anti-CD20 Antibody(NRP-0422-P1230) (Cat#: NRP-0422-P1230)
- NeuroMab™ Anti-TNFα BBB Shuttle Antibody(NRZP-1022-ZP4105) (Cat#: NRZP-1022-ZP4105)
- NeuroMab™ Rabbit Anti-Alpha-synuclein (CBP1631) (Cat#: NAB-08-PZ079)
- NeuroMab™ Anti-FGFR1 Antibody(NRP-0422-P1244) (Cat#: NRP-0422-P1244)
- Mouse Retinal Ganglion Cells (Cat#: NCL2110P145)
- Human Astrocytes, Immortalized (Cat#: NCL-2105-P182-AM)
- Green Fluorescent BACE1 Cell Lines (Cat#: NCL2110P214)
- Human Glial (Oligodendrocytic) Hybrid Cell Line (MO3.13) (Cat#: NCL-2108P34)
- Mouse Microglia Cell Line BV-2, Immortalized (Cat#: NCL2110P153)
- C57 Brain Cortex Neurons [Mouse] (Cat#: NCC20-9PZ48)
- Rat Schwann Cells RSC96, Immortalized (Cat#: NCL-2108P21)
- Human Blood Brain Barrier Model (Cat#: NCL-2103-P187)
- iNeu™ Human Midbrain Dopaminergic Neurons (Cat#: NCL-21P6-003)
- Green Fluorescent Tau SH-SY5Y cell Line (Cat#: NCL2110P219)
- Human GFAP ELISA Kit [Colorimetric] (Cat#: NPP2011ZP383)
- Beta Amyloid (1-42), Aggregation Kit (Cat#: NRZP-0323-ZP200)
- Alpha Synuclein Aggregation Kit (Cat#: NRZP-1122-ZP15)
- Amyloid beta 1-42 Kit (Cat#: NRP-0322-P2170)
- Human Tau Aggregation Kit (Cat#: NRP-0322-P2173)
- Human Poly ADP ribose polymerase,PARP Assay Kit (Cat#: NRZP-1122-ZP62)
- Alpha-Synuclein Aggregation Assay Kit (Cat#: NRZP-1122-ZP37)
- Beta Amyloid (1-40), Aggregation Kit, TTF Assay (Cat#: NRZP-0323-ZP199)
- AAV-mDLX-CRE-tdTomato (Cat#: NRZP-0622-ZP721)
- pAAV-syn-jGCaMP8f-WPRE (Cat#: NTA-2106-P061)
- rAAV-CAG-DIO-G-Flamp1 (Cat#: NRZP-0722-ZP719)
- pAAV-syn-jGCaMP8s-WPRE (Cat#: NTA-2106-P063)
- PRV-CAG-EGFP (Cat#: NTA-2011-ZP14)
- pAAV-syn-FLEX-jGCaMP8m-WPRE (Cat#: NTA-2106-P065)
- rAAV-E-SARE-Cre-ERT2-PEST-WPRE-hGH polyA (Cat#: NTA-2010-TT342)
- VSV-eGFP (Cat#: NTA-2011-ZP20)
- Dextran-CYanine5.5 (Cat#: NTA-2011-ZP118)
- pAAV-syn-jGCaMP8m-WPRE (Cat#: NTA-2106-P062)
- Mouse Parkinson disease (autosomal recessive, early onset) 7 (Park7) (NM_020569) clone, Untagged (Cat#: NEP-0621-R0133)
- Mouse SOD1 shRNA Silencing Adenovirus (Cat#: NV-2106-P14)
- Rat Parkinson disease (autosomal recessive, juvenile) 2, parkin (Park2) (NM_020093) ORF clone/lentiviral particle, Myc-DDK Tagged (Cat#: NEP-0621-R0041)
- 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)
- App Rat amyloid beta (A4) precursor protein (App)(NM_019288) ORF clone, Untagged (Cat#: NEP-0421-R0053)
- Human huntingtin-associated protein 1 (HAP1) transcript variant 2 (NM_177977) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0676)
- Human presenilin 1 (PSEN1), transcript variant 2 (NM_007318) ORF clone, TurboGFP Tagged (Cat#: NEP-0421-R0140)
- Lenti of Mouse synuclein, alpha (Snca) transcript variant (NM_001042451) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0864)
- NeuroBiologics™ Pig Cerebrospinal Fluid (Cat#: NRZP-0822-ZP498)
- NeuroBiologics™ Rat Cerebrospinal Fluid (Cat#: NRZP-0822-ZP496)
- NeuroBiologics™ Human Cerebrospinal Fluid (Cat#: NRZP-0822-ZP491)
- NeuroBiologics™ Mouse Cerebrospinal Fluid (Cat#: NRZP-0822-ZP497)
- NeuroBiologics™ Monkey Cerebrospinal Fluid (Cat#: NRZP-0822-ZP495)
- NeuroPro™ Anti-IDUA BBB Shuttle Protein (Cat#: NRZP-0423-ZP502)
- NeuroPro™ Anti-TNFR BBB Shuttle Protein (Cat#: NRZP-0423-ZP510)
- NeuroPro™ Anti-NAGLU BBB Shuttle Protein (Cat#: NRZP-0423-ZP506)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP500)
- NeuroPro™ Anti-EPO BBB Shuttle Protein (Cat#: NRZP-0423-ZP508)
- NeuroPro™ Anti-ASA BBB Shuttle Protein (Cat#: NRZP-0423-ZP504)
- NeuroPro™ Anti-IDS BBB Shuttle Protein (Cat#: NRZP-0423-ZP503)
- NeuroPro™ Anti-Erythropoietin BBB Shuttle Protein (Cat#: NRZP-0423-ZP499)
- NeuroPro™ Anti-SGSH BBB Shuttle Protein (Cat#: NRZP-0423-ZP505)
- NeuroPro™ Anti-idursulfase BBB Shuttle Protein (Cat#: NRZP-0423-ZP497)