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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Prion protein PrP Mouse Monoclonal Antibody
- Host Species:
- Mouse
- Species Reactivity:
- Cow; Human
- Applications:
- WB; ELISA
- Conjugation:
- Unconjugated
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Prion protein PrP Rabbit Monoclonal Antibody
- Host Species:
- Rabbit
- Species Reactivity:
- Mouse; Rat; Sheep; Cow; Human
- Applications:
- WB; IP
- Conjugation:
- Unconjugated
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Prion protein PrP Mouse Monoclonal Antibody
- Host Species:
- Mouse
- Species Reactivity:
- Sheep; Cow; Human; Deer; Elk
- Applications:
- ELISA; IHC-P; WB; FC
- Conjugation:
- Unconjugated
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Prion protein PrP Mouse Monoclonal Antibody
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Sheep; Cow; Human; Monkey
- Applications:
- IHC-P; WB; IP; ICC; FC; Indirect 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 2040
![](static/images/product/product-icon-neural-antibodies.png)
Prion protein PrP Rabbit Monoclonal Antibody
- Host Species:
- Rabbit
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; FC; IHC-P
- 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 2038
- NeuroMab™ Anti-Tau Antibody(NRP-0422-P1686) (Cat#: NRP-0422-P1686)
- NeuroMab™ Anti-F-Spondin/SPON1 Antibody, Clone N24875P (CBP11839) (Cat#: NRZP-0822-ZP4740)
- NeuroMab™ Rabbit Anti-Alpha-synuclein (CBP1631) (Cat#: NAB-08-PZ079)
- NeuroMab™ Anti-ApoC3 BBB Shuttle Antibody(NRZP-1022-ZP3503) (Cat#: NRZP-1022-ZP3503)
- NeuroMab™ Mouse Anti-LRP1 Monoclonal Antibody (CBP3363) (Cat#: NAB-0720-Z6479)
- NeuroMab™ Rabbit Anti-LRRK2 Monoclonal Antibody (CBP1887) (Cat#: NAB-08-PZ735)
- NeuroMab™ Anti-PVRIG Antibody(NRP-0422-P517) (Cat#: NRP-0422-P517)
- NeuroMab™ Anti-PVRIG Antibody(NRP-0422-P515) (Cat#: NRP-0422-P515)
- NeuroMab™ Anti-GD2 Antibody(NRZP-1222-ZP767) (Cat#: NRZP-1222-ZP767)
- NeuroMab™ Anti-PVRIG Antibody(NRP-0422-P792) (Cat#: NRP-0422-P792)
- Mouse Glioma Cell Line GL261-GFP (Cat#: NCL-2108P04)
- iNeu™ Human Neural Stem Cell Line (Cat#: NCL200552ZP)
- Human Astrocytes (Cat#: NCC20-9PZ01)
- Human Dental Pulp Stem Cells (Cat#: NRZP-1122-ZP113)
- Mouse Hippocampal Neuron Cell HT22 (Cat#: NCL2110P001)
- iNeu™ Human Motor Neurons (Cat#: NCL-2103-P71)
- C57 Brain Cortex Neurons [Mouse] (Cat#: NCC20-9PZ48)
- Human Glioblastoma Cell Line SF126 (Cat#: NCL-2108P35)
- Human CMEC/D3 Cell Line, Blood-Brain Barrier Model (Cat#: NCL-2108-P020)
- Human Astrocytes, Immortalized (Cat#: NCL-2105-P182-AM)
- Beta Amyloid (1-40), Aggregation Kit, TTF Assay (Cat#: NRZP-0323-ZP199)
- Alpha Synuclein Aggregation Kit (Cat#: NRZP-1122-ZP15)
- Beta Amyloid (1-42), Aggregation Kit (Cat#: NRZP-0323-ZP200)
- Alpha-Synuclein Aggregation Assay Kit (Cat#: NRZP-1122-ZP37)
- Human GFAP ELISA Kit [Colorimetric] (Cat#: NPP2011ZP383)
- Human Tau Aggregation Kit (Cat#: NRP-0322-P2173)
- Human Poly ADP ribose polymerase,PARP Assay Kit (Cat#: NRZP-1122-ZP62)
- Amyloid beta 1-42 Kit (Cat#: NRP-0322-P2170)
- PRV-CAG-EGFP (Cat#: NTA-2011-ZP14)
- AAV2 Full Capsids, Reference Standards (Cat#: NTC2101070CR)
- pAAV-syn-jGCaMP8m-WPRE (Cat#: NTA-2106-P062)
- Dextran-FITC (Cat#: NTA-2011-ZP110)
- AAV-EF1a-mCherry-flex-dtA (Cat#: NRZP-0622-ZP616)
- Dextran, NHS Activated, 40 kDa (Cat#: NRZP-0722-ZP124)
- Dextran-CYanine5.5 (Cat#: NTA-2011-ZP118)
- Dextran, Cy5 Labeled, 2000 kDa (Cat#: NRZP-0722-ZP22)
- pAAV-syn-FLEX-jGCaMP8s-WPRE (Cat#: NTA-2106-P066)
- pAAV-hSyn-DIO-XCaMP-R-WPRE (Cat#: NTA-2012AD-P508)
- Rat Parkinson disease (autosomal recessive, juvenile) 2, parkin (Park2) (NM_020093) ORF clone/lentiviral particle, Myc-DDK Tagged (Cat#: NEP-0621-R0041)
- Tau Antisense Oligonucleotide (IONIS-MAPTRx) (Cat#: NV-2106-P29)
- Human presenilin 1 (PSEN1), transcript variant 2 (NM_007318) ORF clone, TurboGFP Tagged (Cat#: NEP-0421-R0140)
- Human superoxide dismutase 1, soluble (SOD1) (NM_000454) ORF clone, TurboGFP Tagged (Cat#: NEP-0521-R0748)
- ABCA1 Antisense Oligonucleotide (NV-2106-P27) (Cat#: NV-2106-P27)
- Lenti of Mouse synuclein, alpha (Snca) transcript variant (NM_001042451) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0864)
- Mouse SOD1 shRNA Silencing Adenovirus (Cat#: NV-2106-P14)
- Human apolipoprotein E (APOE) (NM_000041) ORF clone, Untagged (Cat#: NEP-0421-R0232)
- Human huntingtin-associated protein 1 (HAP1) transcript variant 2 (NM_177977) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0676)
- Human superoxide dismutase 3, extracellular (SOD3) (NM_003102) ORF clone, Untagged (Cat#: NEP-0521-R0808)
- 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-PON1 BBB Shuttle Protein (Cat#: NRZP-0423-ZP507)
- NeuroPro™ Anti-SGSH BBB Shuttle Protein (Cat#: NRZP-0423-ZP505)
- NeuroPro™ Anti-IDS BBB Shuttle Protein (Cat#: NRZP-0423-ZP503)
- NeuroPro™ Anti-TNFR BBB Shuttle Protein (Cat#: NRZP-0423-ZP510)
- NeuroPro™ Anti-ASA BBB Shuttle Protein (Cat#: NRZP-0423-ZP504)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP509)
- NeuroPro™ Anti-NAGLU BBB Shuttle Protein (Cat#: NRZP-0423-ZP506)
- NeuroPro™ Anti-TNFR BBB Shuttle Protein (Cat#: NRZP-0423-ZP501)
- NeuroPro™ Anti-idursulfase BBB Shuttle Protein (Cat#: NRZP-0423-ZP497)