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C9orf72 Gene-Engineered Cell Models Products

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C9orf72 is a gene critically involved in neurodegenerative diseases, notably frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Pathogenic mutations in C9orf72, such as the hexanucleotide repeat expansion (HRE), result in a complex pathogenic mechanism. This involves a combination of loss of normal gene function and gain of toxic, aberrant functions, ultimately driving the characteristic disease pathology. Consequently, accurate cell models are indispensable tools. They are essential for dissecting the complexities of these diseases and for developing effective therapeutic strategies. Creative Biolabs offers a comprehensive suite of meticulously engineered C9orf72 Gene-Engineered Cell Model Products, providing researchers with reliable tools that deliver consistent, reproducible results. Contact us for transformative tools!

You can explore our C9orf72 model categories below, or view our complete Product List.

Featured C9orf72 Gene-Engineered Cell Model Categories

We offer a variety of C9orf72 cell models, categorized by their specific modifications:

C9orf72 Mutation Models: These models have specific C9orf72 gene changes. Scientists see these changes in people with ALS and FTD. The models are useful. They help study exactly how these changes affect the protein. You can learn how the protein works. You can also learn how it processes RNA. The models also help study other cell processes. For example, these models can use the pathogenic GGGGCC repeat expansion. This happens in the non-coding part of the C9orf72 gene. It is the most common cause of ALS and FTD. This repeat can happen many times. There can be tens to thousands of repeats. Scientists study this a lot. You can also add other changes to the models. It depends on what you need to study.
C9orf72 Knock-in Models: These models allow for the insertion of the C9orf72 gene into a specific location in the cell's DNA, enabling controlled studies of C9orf72 expression and function. They are particularly useful for examining how disease-related changes affect C9orf72 interactions, localization, and RNA processing.
C9orf72 Knock-out Models: In these models, the C9orf72 gene is turned off, preventing the production of the C9ORF72 protein. These models help elucidate the normal functions of C9orf72 and the consequences of its absence, such as alterations in RNA processing, stress granules, and autophagy. They can also aid in the identification of new drug targets.

Features of Our C9orf72 Gene-Engineered Cell Models

Our C9orf72 Gene-Engineered cell models are characterized by:

Feature	Description
Precise Gene Modification	We utilize cutting-edge gene-editing technologies to introduce specific C9orf72 alterations with high accuracy, minimizing off-target effects.
Disease Relevance	Our models replicate key aspects of C9orf72 proteinopathies, closely mimicking the cellular and molecular changes observed in ALS, FTLD, and other neurodegenerative diseases.
Versatility	We offer a wide range of models, including knockout, knock-in, and overexpression, to accommodate diverse research objectives and experimental designs.

Advantages of Our C9orf72 Gene-Engineered Cell Models

Reliable disease models: Our cell models are made to copy the gene and trait changes of C9orf72 brain diseases. So, they give a strong base for your research. This means your studies use a good model. This leads to results you can trust and that have a big impact.
Accelerated research timelines: We give you cell models that are ready to use and well-studied. This saves you time. You don't have to make and check your own models. So, you can focus on your main research questions. You can also make progress faster in finding new things and ways to treat diseases.
Customizable solutions: We know each research project is different. So, we let you change our models. You can pick cell types, gene backgrounds, and other things. This lets you create a model that fits your exact experiment. This way, you have the best tools to solve your specific research problems.
Expert support: Our team knows a lot about science. We will give you full support as you do your research. We help you from the start to when you look at your data. This makes sure you can use our cell models well. It also helps you reach your research goals.

A picture presents the C9ORF72 gene structure, transcript variants, and protein isoforms. (OA Literature)

Fig.1 C9ORF72 gene structure, transcript variants, and protein isoforms under a non-pathological (A) and a pathological (B) state.¹

C9ORF72 Gene: Mechanisms and Principles

The C9orf72 gene contains a repeating DNA sequence (G4C2). When this repeat expands, it causes changes in DNA, RNA, and protein. The C9orf72 HRE can form G-quadruplexes and R-loops. These structures block RNA polymerase, leading to truncated transcripts and loss of normal transcripts. The repeat-containing RNAs trap proteins, causing stress. The C9orf72 mutation causes both loss of normal function and gain of toxic effects from RNA and dipeptide repeat proteins (DPRs), contributing to ALS and FTD.

FAQs

How can these cell models help my research?
These cell models are versatile tools for your research. They enable in-depth study of C9orf72-related neurodegenerative disease mechanisms. This is crucial for understanding these conditions. They also offer a platform to screen drugs for efficacy and safety. Ultimately, they aid in developing targeted treatments addressing the core molecular issues of ALS/FTD.
What is a hexanucleotide repeat expansion (HRE)?
In the C9orf72 gene, HRE means a specific gene change. This change is called a hexanucleotide repeat expansion (HRE). In HRE, a DNA sequence (GGGGCC) is repeated too many times. This happens much more than normal. This repeat problem is a key sign of ALS and FTD.
What other C9orf72 mutations can be modeled?
Besides HRE, our models include other C9orf72 gene changes. These are point mutations, where a single DNA letter changes. This can alter the protein's building blocks (amino acids). Examples are p.Arg116Gly, p.Asp91Ala, and p.Leu145Phe. These specific mutations occur in people with brain diseases.
What cell types are available for your C9orf72 gene-engineered models?
We offer different C9orf72 cell models. These include nerve cells (neuronal), glial cells, and fibroblast cells. You can also get custom cell lines. This lets you study how C9orf72 changes affect various cells. Contact us to discuss your specific research needs.
How do I get started with ordering or requesting a custom cell model?
To start ordering or requesting a custom cell model, you have two options. You can use our website to submit an online quote. Or, you can email us at info@creative-biolabs.com. This lets you set up a meeting. At the meeting, you can talk about what you need for your project. We can discuss your experiment design and the exact cell model you want. If needed, we can also talk about making a custom model for you.

At Creative Biolabs, our commitment lies in equipping our clients with C9orf72 Gene-Engineered Cell Model Products of the highest caliber, complemented by unparalleled customer support. We recognize the critical importance of reliable and physiologically relevant models in neurodegenerative disease research. Therefore, we provide meticulously generated cell lines and comprehensive assistance to ensure your project's success. For further information regarding our C9orf72 cell model portfolio or to discuss the specific requirements of your research endeavors, we encourage you to contact our dedicated team.