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Neurocutaneous Syndrome Related 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. Possible contributors and molecular pathways involved in the three common neurocutaneous disorders are shown in Fig.1.

Fig.1 The scheme of the RAS/MAPK and mTOR pathways. (Stafstrom, 2017)

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.

Reference

Stafstrom, C. E.; et al. Epilepsy mechanisms in neurocutaneous disorders: tuberous sclerosis complex, neurofibromatosis type 1, and Sturge-Weber syndrome. Frontiers in neurology. 2017, 8: 87.

For Research Use Only. Not For Clinical Use.

Target

Il-1β

Alpha Internexin

LRRK2

FGGY

FDFT1

HIP7

Laminin gamma 3

Neurabin 1

APPBP2

STAT3 Phospho (Ser727)

Syntaxin 3

CAV1.2

NOTCH2

Arc

Neuroligin 3

GAD1

Insulin Degrading Enzyme

ASPA

Cathepsin D

CD271

Stathmin 1

PRMT1

CRMP4

PAX5

Neurofilament L

M1 Muscarinic Receptor

FABP3

TrkA

Flt1

14-3-3 alpha/beta

Huntingtin

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Host Species:: Rabbit

Species Reactivity:: Human

Applications:: IHC-P

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