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Nervous System


Neurological disease is a rising burden for society and finance in aging societies, impacting approximately 1 billion individuals around the world, especially regarding neurodegenerative diseases including dementia, Alzheimer’s disease (AD), and Parkinson’s diseases (PD). The molecular basis of most neurodegenerative diseases is not fully understood till now, and the study of neurological disorders is particularly challenging. The emerging technology of iPSC attracts attention worldwide, giving access to genetic/epigenetic mechanism studies and new drug/methods exploration of neurodegenerative disease by generating patient-specific disease models in vitro. Creative Biolabs provides a series of patient-specific iPSC models of genetically inherited neurological diseases, which will satisfy different research demands for worldwide clients.

Together with gene editing techniques to reprogram human somatic cell into iPSC and differentiate iPSC into neurons, iPSC-based neurological disease models have revolutionized the utility of human in vitro models of neurological disease in the past decade, contributing to molecular/pathological mechanisms understanding, early diagnosis, drug discovery/screening as well as personalized precision medicine. Experienced ability is critical for generating iPSC-based models, including generating, expanding and purifying functional and disease-relevant neurological cells.

The Generation Steps of hPSC-based Disease Models

Neurological Lineages Differentiation of hPSCs

Patients’ somatic cells like fibroblasts and other peripheral cells are collected and reprogramed into human pluripotent stem cells (hPSCs). Differentiation of hPSCs is regulated by particular cellular factors, mainly belonging to four signaling pathways (BMP, FGF, WNT/β-catenin and TGF-β/activin/nodal). Thus far, hPSCs have been successfully differentiated into cortical neurons, DA neurons, motor neurons, astrocyte, Purkinje cell, neural progenitor cells (NPCs) as well as neural stem cells (NSCs) by researchers around the world.

Directed Neural Differentiation of Human Pluripotent Stem Cells Fig 1. Directed Neural Differentiation of Human Pluripotent Stem Cells.

Features of Nervous System Disease Models

hPSC Models of Neurological Diseases

hPSC-neurons (hPSC-NU) models of inherited neurological diseases are used to investigate neurological disease pathology mechanism and evaluate new medical methods and drugs. Creative Biolabs has established a state-of-the-art STEMODTM neurological disease models platform, to offer a list of hPSC-UN models with accurate evaluated neurological disease phenotypes. Those models are prepared for the investigation of Alzheimer’s disease, Parkinson’s disease and other inherited neurodegenerative disorders. Please feel free to contact us if you don’t find the proper neurological disease models for your research. Customized service will also be provided to meet every specific demand!

References

  1. Chang, C.; et al. (2018). Induced Pluripotent Stem Cells. Cell Transplantation, 27(11), pp.1588-1602.
  2. Park, I.; et al. (2008). Disease-Specific Induced Pluripotent Stem Cells. Cell, 134(5), pp.877-886.
  3. Mungenast, A.; et al. (2016). Modeling Alzheimer's disease with human induced pluripotent stem (iPS) cells. Molecular and Cellular Neuroscience, 73, pp.13-31.

For Lab Research Use Only, Not for Human or Animal Therapeutic Use.

Datasheet INQUIRY
Target Gene: PSEN1
Mutation: A246E
Organ System: Central Nervous System
Disease: Alzheimer's Disease (AD)
Datasheet INQUIRY
Target Gene: PSEN1
Mutation: M146V
Organ System: Central Nervous System
Disease: Alzheimer's Disease (AD)
Datasheet INQUIRY
Target Gene: PSEN2
Mutation: N141I
Organ System: Central Nervous System
Disease: Alzheimer's Disease (AD)
Datasheet INQUIRY
Target Gene: APP
Mutation: ΔE693
Organ System: Central Nervous System
Disease: Alzheimer's Disease (AD)
Datasheet INQUIRY
Target Gene: APP
Mutation: V717L
Organ System: Central Nervous System
Disease: Alzheimer's Disease (AD)
Datasheet INQUIRY
Target Gene: APP
Mutation: APPDp
Organ System: Central Nervous System
Disease: Alzheimer's Disease (AD)
Datasheet INQUIRY
Target Gene: APP
Mutation: A673T
Organ System: Central Nervous System
Disease: Alzheimer's Disease (AD)
Datasheet INQUIRY
Target Gene: SNCA
Mutation: SNCA_Tri
Organ System: Central Nervous System
Disease: Parkinson Disease (PD)
Datasheet INQUIRY
Target Gene: Parkin (PRAK2)
Mutation: Exon 2, 4 homozygous deletions
Organ System: Central Nervous System
Disease: Parkinson Disease (PD)
Datasheet INQUIRY
Target Gene: Parkin (PRAK2)
Mutation: Exon 6, 7 homozygous deletions
Organ System: Central Nervous System
Disease: Parkinson Disease (PD)
Datasheet INQUIRY
Target Gene: Parkin (PRAK2)
Mutation: Exon 3 homozygous deletions
Organ System: Central Nervous System
Disease: Parkinson Disease (PD)
Datasheet INQUIRY
Target Gene: Parkin (PRAK2)
Mutation: Exon 3, 5 homozygous deletions
Organ System: Central Nervous System
Disease: Parkinson Disease (PD)
Datasheet INQUIRY
Target Gene: Parkin (PRAK2)
Mutation: V324A
Organ System: Central Nervous System
Disease: Parkinson Disease (PD)
Datasheet INQUIRY
Target Gene: PINK1 (PRAK6)
Mutation: Q456X
Organ System: Central Nervous System
Disease: Parkinson Disease (PD)
Datasheet INQUIRY
Target Gene: PINK1 (PRAK6)
Mutation: c.1488 + 1G> A + c.1252_1488
Organ System: Central Nervous System
Disease: Parkinson Disease (PD)
Datasheet INQUIRY
Target Gene: PINK1 (PRAK6)
Mutation: G309D (c.926G> A)
Organ System: Central Nervous System
Disease: Parkinson Disease (PD)
Datasheet INQUIRY
Target Gene: TARDBP
Mutation: M337V
Organ System: Central Nervous System
Disease: Amyotrophic Lateral Sclerosis (ALS)
Datasheet INQUIRY
Target Gene: TARDBP
Mutation: Q343R
Organ System: Central Nervous System
Disease: Amyotrophic Lateral Sclerosis (ALS)
Datasheet INQUIRY
Target Gene: TARDBP
Mutation: G298S
Organ System: Central Nervous System
Disease: Amyotrophic Lateral Sclerosis (ALS)
Datasheet INQUIRY
Target Gene: FUS
Mutation: R521H
Organ System: Central Nervous System
Disease: Amyotrophic Lateral Sclerosis (ALS)

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