Neurocutaneous Syndrome Research Tools
Neurocutaneous syndromes represent a heterogeneous group of genetic disorders characterized by abnormalities of the cutaneous and nervous systems. Neurofibromatosis type I (NF1), Tuberous sclerosis complex (TSC), and Sturge-Weber syndrome (SWS) are the three prototypical neurocutaneous disorders in which genetic mutations in the pathways regulating cell growth cause developmental dysfunctions of the brain, skin, and other organs.
The mTOR signaling pathway begins at the cell membrane, where the receptors respond to growth factors and nutrient/energy molecules. Phosphatidylinositol triphosphate 3-kinase (PI3K) activates protein kinase B (AKT), a serine/threonine-specific protein kinase, and potent pro-survival and pro-oncogenic proteins. AKT directly phosphorylates TSC2 and inhibits its function. TSC2 inactivation by AKT reduces Ras homolog enriched in brain (Rheb). Overexpression of Rheb due to aTSC1 or TSC2 mutation leads to increased mTOR activation and excessive cell growth and proliferation.
Summary of the Popular Objects in Neurocutaneous Syndromes
Based on both unique molecular pathways for each syndrome and commonalities among syndromes, targeted therapies are emerging from the understanding of neurocutaneous disorders at the molecular level. Some key factors that may lead to neuronal hyperexcitability in these neurocutaneous disorders are listed in the table below.
| PLC | PKC | Ras | Raf |
| TSC1 | TSC2 | NF1 | NF2 |
| MEK | ERK | GANQ | ELFN1 |
| PI3K | AKT | Rheb | FKBP38 |
Creative Biolabs offers comprehensive products as well as high-quality customized services for our clients, such as neural cell lines, specialty media, cytokines, small molecule inhibitors, recombinant proteins, ready-to-use antibodies or commercial products, etc. We also provide animal models for our clients. We have the best products for the popular targets to aid your disease research.
For Research Use Only. Not For Clinical Use.
Target
Related Products
Loading...
iNeuMab™ Rabbit Anti-NCAN Monoclonal Antibody (CBP7291) [CAT#: NAB-0720-Z9943]
Neurocan Rabbit Monoclonal Antibody
- Host Species:
- Rabbit
- Species Reactivity:
- Human
- Applications:
- IHC-P
- Conjugation:
- Unconjugated
iNeuMab™ Rabbit Anti-LAMC3 Monoclonal Antibody (CBP7248) [CAT#: NAB-0720-Z9832]
Laminin gamma 3 Rabbit Monoclonal Antibody
- Host Species:
- Rabbit
- Species Reactivity:
- Mouse; Rat
- Applications:
- WB; IHC-P; IHC-Fr
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-Rabbit CACNA1C Monoclonal Antibody (CBP6892) [CAT#: NAB2109P238]
Anti-CAV1.2 Monoclonal Antibody
- Host Species:
- Mouse
- Species Reactivity:
- Rabbit
- Applications:
- IF; WB; IHC; IP
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-APOE Monoclonal Antibody (CBP6535) [CAT#: NAB-1021-R0405]
Mouse monoclonal to APOE
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- WB; ELISA
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-PRMT1 Monoclonal Antibody (CBP6262) [CAT#: NAB-1021-R0117]
Mouse monoclonal to PRMT1
- Host Species:
- Mouse
- Species Reactivity:
- Human; Mouse; Rat
- Applications:
- WB; IF; IHC-P
- Conjugation:
- Unconjugated
iNeuMab™ Rabbit Anti-VEGFR1 Monoclonal Antibody (CBP6098) [CAT#: NAB-BA-P3976]
Predicted cross reactivity: Rat
- Host Species:
- Rabbit
- Species Reactivity:
- Human; Mouse
- Applications:
- WB; IHC-P; IHC-F; ICC; IF
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-HIP7 Monoclonal Antibody (CBP6056) [CAT#: NAB-0421-R0639]
Mouse monoclonal to HIP7
- Host Species:
- Mouse
- Species Reactivity:
- Human; Mouse; Rat
- Applications:
- IHC; WB
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-GAD1 Monoclonal Antibody (CBP6012) [CAT#: NAB-0421-R0460]
Mouse monoclonal to GAD1
- Host Species:
- Mouse
- Species Reactivity:
- Human; Mouse; Rat
- Applications:
- IHC; IP; WB
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-FABP3 Monoclonal Antibody (CBP5973) [CAT#: NAB-0421-R0358]
Mouse monoclonal to FABP3
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-NEFL Monoclonal Antibody (CBP5943) [CAT#: NAB-0421-R0304]
Mouse monoclonal to NEFL
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-GFAP Monoclonal Antibody (CBP5903) [CAT#: NAB-0421-R0160]
Mouse monoclonal to GFAP
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-GFAP Monoclonal Antibody (CBP5878) [CAT#: NAB-0421-R0134]
Mouse monoclonal to GFAP
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-UCHL1 Monoclonal Antibody (CBP5852) [CAT#: NAB-0421-R0077]
Mouse monoclonal to UCHL1
- Host Species:
- Mouse
- Species Reactivity:
- Bovine; Human; Rat
- Applications:
- WB
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-UCHL1 Monoclonal Antibody (CBP5825) [CAT#: NAB-0421-R0016]
Mouse monoclonal to UCHL1
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-IL1B Monoclonal Antibody (CBP5320) [CAT#: NAB-0321-R0787]
Mouse Monoclonal to IL1B
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- IF; IHC; WB
- Conjugation:
- Unconjugated
iNeuMab™ Mouse Anti-APOE Monoclonal Antibody (CBP5266) [CAT#: NAB-0321-R0598]
Mouse Monoclonal to APOE
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- IHC; WB
- Conjugation:
- Unconjugated; APC; PE; HRP; Biotin; FITC
iNeuMab™ Mouse Anti-α-internexin Monoclonal Antibody (CBP5227) [CAT#: NAB-2103-P1143]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; IHC-P; ELISA
iNeuMab™ Mouse Anti-VGAT Monoclonal Antibody (CBP5200) [CAT#: NAB-2103-P1090]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA
iNeuMab™ Mouse Anti-Trk Monoclonal Antibody (CBP5166) [CAT#: NAB-2103-P1023]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; IHC-P; ELISA
iNeuMab™ Mouse Anti-ST Monoclonal Antibody (CBP5124) [CAT#: NAB-2103-P958]
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- WB; IP; IF; ELISA
iNeuMab™ Mouse Anti-PRNPIP Monoclonal Antibody (CBP5055) [CAT#: NAB-2103-P826]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA
iNeuMab™ Mouse Anti-Pancreatic Monoclonal Antibody (CBP5014) [CAT#: NAB-2103-P761]
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- WB; IP; IF; ELISA
iNeuMab™ Mouse Anti-NPY Receptor Monoclonal Antibody (CBP4983) [CAT#: NAB-2103-P711]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA
iNeuMab™ Mouse Anti-NGFR Monoclonal Antibody (CBP4947) [CAT#: NAB-2103-P661]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; IHC-P
iNeuMab™ Mouse Anti-Neuroserpin Monoclonal Antibody (CBP4915) [CAT#: NAB-2103-P604]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA
iNeuMab™ Mouse Anti-Neurabin Monoclonal Antibody (CBP4886) [CAT#: NAB-2103-P557]
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- WB; IP; IF; IHC-P; ELISA
iNeuMab™ Mouse Anti-Myelin Basic Protein Monoclonal Antibody (CBP4856) [CAT#: NAB-2103-P505]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA
iNeuMab™ Mouse Anti-mAChR M Monoclonal Antibody (CBP4821) [CAT#: NAB-2103-P453]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA
iNeuMab™ Mouse Anti-Huntingtin Monoclonal Antibody (CBP4792) [CAT#: NAB-2103-P399]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; IHC-P
iNeuMab™ Mouse Anti-GRIP Monoclonal Antibody (CBP4764) [CAT#: NAB-2103-P353]
- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA
Products
Hot Products
Antibodies
- iNeuMab™ Mouse Anti-EFNB2 Monoclonal Antibody (CBP1159) (Cat#: NAB-0720-Z4396)
- Mouse Anti-Human α-Synuclein Phospho (Tyr39) (CBP3706) (Cat#: NAB201250LS)
- iNeuMab™ Anti-F-Spondin/SPON1 Antibody, Clone 3F4 (Cat#: NRZP-0822-ZP4740)
- iNeuMab™ Mouse Anti-LRP1 Monoclonal Antibody (CBP3363) (Cat#: NAB-0720-Z6479)
- iNeuMab™ Rabbit Anti-LRRK2 Monoclonal Antibody (CBP1887) (Cat#: NAB-08-PZ735)
- Mouse Anti-SCN5A Monoclonal Antibody (CBP708) (Cat#: NAB-0720-Z2720)
- iNeuMab™ Mouse Anti-SHANK3 Monoclonal Antibody (CBP929) (Cat#: NAB-0720-Z3477)
- iNeuMab™ Rabbit Anti-Alpha-synuclein (CBP1631) (Cat#: NAB-08-PZ079)
More
Cells
- Mouse Glioma Cell Line GL261 (Cat#: NCL-2108P28)
- Immortalized Human Cerebral Microvascular Endothelial Cells (Cat#: NCL-2108-P020)
- Green Fluorescent Tau cell Line (Cat#: NCL2110P219)
- Human Brain Vascular Adventitial Fibroblasts (Cat#: NCL-21P6-014)
- Mouse Glioma Cell Line GL-261-Luc (Cat#: NCL-2108P06)
- Mouse Retinal Ganglion Cells (Cat#: NCL2110P145)
- Mouse Midbrain Dopaminergic Neuron Cell MN9D (Cat#: NCL2110P059)
- Mouse Glioma Cell Line GL261-GFP (Cat#: NCL-2108P04)
- iNeu™ Human Motor Neurons (Cat#: NCL-2103-P71)
- Mouse Microglia N9 (Cat#: NCL2110P073)
More
Kits
- Alpha-Synuclein Aggregation Assay Kit (Cat#: NRZP-1122-ZP37)
- Alpha Synuclein Aggregation Kit (Cat#: NRZP-1122-ZP15)
- Beta Amyloid (1-40), Aggregation Kit (Cat#: NRZP-0323-ZP199)
- Beta Amyloid (1-42), Aggregation Kit (Cat#: NRZP-0323-ZP200)
- Amyloid beta 1-42 Kit (Cat#: NRP-0322-P2170)
- Human GFAP ELISA Kit [Colorimetric] (Cat#: NPP2011ZP383)
- Human Poly ADP ribose polymerase,PARP Assay Kit (Cat#: NRZP-1122-ZP62)
- Human Tau Aggregation Kit (Cat#: NRP-0322-P2173)
More
Tracers
- VSV-eGFP (Cat#: NTA-2011-ZP20)
- AAV2 Full Capsids, Reference Standards (Cat#: NTC2101070CR)
- Dextran, NHS Activated (Cat#: NRZP-0722-ZP124)
More
Gene Therapy Tools
- Lenti of Human TAR DNA binding protein (TARDBP) (NM_007375) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0832)
- Human superoxide dismutase 3, extracellular (SOD3) (NM_003102) ORF clone, Untagged (Cat#: NEP-0521-R0808)
- Human superoxide dismutase 1, soluble (SOD1) (NM_000454) ORF clone, TurboGFP Tagged (Cat#: NEP-0521-R0748)
- Rat Parkinson disease (autosomal recessive, juvenile) 2, parkin (Park2) (NM_020093) ORF clone/lentiviral particle, Myc-DDK Tagged (Cat#: NEP-0621-R0041)
- Human huntingtin (HTT) (NM_002111) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0497)
- Human presenilin 1 (PSEN1), transcript variant 2 (NM_007318) ORF clone, TurboGFP Tagged (Cat#: NEP-0421-R0140)
- Mouse SOD1 shRNA Silencing Adenovirus (Cat#: NV-2106-P14)
- Tau Antisense Oligonucleotide (Cat#: NV-2106-P29)
- Lenti of Mouse synuclein, alpha (Snca) transcript variant (NM_001042451) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0864)
- Human apolipoprotein E (APOE) (NM_000041) ORF clone, Untagged (Cat#: NEP-0421-R0232)
More
Biospecimens
- NeuroBiologics™ Pig Cerebrospinal Fluid (Cat#: NRZP-0822-ZP498)
- NeuroBiologics™ Monkey Cerebrospinal Fluid (Cat#: NRZP-0822-ZP495)
- NeuroBiologics™ Human Cerebrospinal Fluid (Cat#: NRZP-0822-ZP491)
- NeuroBiologics™ Mouse Cerebrospinal Fluid (Cat#: NRZP-0822-ZP497)
- NeuroBiologics™ Rat Cerebrospinal Fluid (Cat#: NRZP-0822-ZP496)
More
Cell Assay Reagents
- NeuroPro™ Anti-idursulfase BBB Shuttle Protein (Cat#: NRZP-0423-ZP497)
- NeuroPro™ Anti-IDS BBB Shuttle Protein (Cat#: NRZP-0423-ZP503)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP509)
- NeuroPro™ Anti-EPO BBB Shuttle Protein (Cat#: NRZP-0423-ZP508)
- NeuroPro™ Anti-Erythropoietin BBB Shuttle Protein (Cat#: NRZP-0423-ZP499)
- NeuroPro™ Anti-IDUA BBB Shuttle Protein (Cat#: NRZP-0423-ZP498)
- NeuroPro™ Anti-NAGLU BBB Shuttle Protein (Cat#: NRZP-0423-ZP506)
- NeuroPro™ Anti-IDUA BBB Shuttle Protein (Cat#: NRZP-0423-ZP502)
- NeuroPro™ Anti-ASA BBB Shuttle Protein (Cat#: NRZP-0423-ZP504)
- NeuroPro™ Anti-PON1 BBB Shuttle Protein (Cat#: NRZP-0423-ZP507)
More
