iNeu™ Human Motor Neuron Progenitors- Amyotrophic Lateral Sclerosis (ALS) (C9ORF72 expansion)

Species:: Human

Cell Types:: Motor Neurons

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Product Overview

Description

C9orf72 is present in ~ 40% of familial ALS and 8-10 % of sporadic ALS. It is the most common mutation related to ALS - far more common than SOD1 or TDP-43.

Creative Biolabs's Human iPSC-Derived Motor Neuron Progenitors from a female donor diagnosed with amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), carrying C9orf72 hexanucleotide expansion.

Neurological Disease Models

ALS-related Cells

Cell Types

Motor Neurons

Mutation Description

C9ORF72 repeat expansion

Relevant Diseases

Amyotrophic Lateral Sclerosis

Species

Human

Properties

Size

≥2 x 106 cells

Tissue Source

Fibroblasts (64 yr Female)

Cell Purity

>95%

Cell Viability

>90%

Mycoplasma Testing

The cell line has been screened using the luciferase based mycoplasma detection kit to confirm the absence of mycoplasma species.

Sterility Testing

Sterility testing was performed in accordance with USP and EP regulations. All of our sterility testing is performed in an isolator or clean room environments. The cell line has been screened using the membrane filtration testing methods to confirm the absence of aerobic, anaerobic and fungi microorganisms.

Genetic Stability Testing

Cell genetic stability study was perfomed under ICH guidelines. We provide guidance on the appropriate testing program upon your requirements.

Shipping

Dry ice

Storage

Liquid nitrogen

Handling Advice

Frozen cells:Upon receipt, frozen ampoules should be transferred directly to gaseous phase liquid nitrogen without delay, unless they are to be used straight away.

Growing cells: Growing cell cultures should be checked on receipt using an inverted microscope. Immediately check the cell density upon arrival for any obvious defects. If the cell density is too high (more than 80% confluent) subculture the cells (harvest and reseed) immediately.

For detailed instructions on the thawing procedure of frozen cells and the culture of adherent or suspended cells, please feel free to contact us by email or phone.

Research Use Only

For research use only, not for diagnostic or therapeutic use.

Warnings

Store under recommended storage conditions (liquid nitrogen). Do not expose to high temperature. After expiration, discard all remaining reagents. It is recommended to use cells within ten generations.

Quality Control

Each batch of cells has passed the high-level expression of Nestin and Sox 2 as well as its self-renewal and multi-lineage differentiation ability. The cells also showed normal karyotypes, and mycoplasma tests were negative by chromosome spread assessment. The blood of pregnant women has been screened for HIV, HTLV, hepatitis B and C.

Donor Information

Donor Health

Disease

Ethnicity

Caucasian (Ashkenazi)

Additional Donor Information

Confirmed ALS

Publications

Publications (0)

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Scientific Resources

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Customer Reviews and Q&As

Customer Reviews Average Customer Ratings Overall

5.0

Excellent

Well-characterized nature

The cells are highly reproducible and demonstrate the expected pathological features associated with ALS. Our experiments on neurodegeneration pathways have yielded promising results thanks to the high quality of these progenitors.

Excellent

The cells exhibit the necessary disease-specific characteristic

The cells show clear disease phenotypes, and their consistency has helped in validating our hypotheses. An excellent model for ALS research.

Excellent

The progenitor cells are of high quality

These motor neuron progenitors provide an effective model for investigating ALS mechanisms, particularly those related to the C9ORF72 expansion. They are well-characterized and robust, which has streamlined our experimental workflows and data collection.

Excellent

Offered a reliable model of ALS

The quality and integrity of the cells allow for reproducible experiments and significant insights into disease pathology.

Excellent

They exhibit the disease-related features

We have been using these progenitors for several months and have observed consistent and reproducible ALS-related phenotypes.

Q&As

Are there any specific protocols or recommendations for differentiating these progenitors into mature motor neurons?

We provide detailed protocols for differentiating these progenitors into mature motor neurons. The process typically involves a series of differentiation steps using specific growth factors and signaling molecules. We recommend following our protocol closely and optimizing conditions based on your experimental needs to achieve the desired maturation of motor neurons.

What types of assays can be performed using these progenitors to study ALS pathology?

Various assays can be performed, including immunostaining to detect disease-specific markers, western blotting for protein analysis, and live-cell imaging to monitor cellular processes. Additionally, you can conduct assays to assess neurodegeneration, cell viability, and the effects of potential therapeutic agents.

What support or resources do you provide?

We offer comprehensive support, including detailed product information, differentiation protocols, and technical assistance. Our team is available to address any questions or challenges you may encounter. We also provide updates on best practices and troubleshooting tips to ensure successful implementation in your research.