C9orf72 Knockout mouse models
Faithful ALS and frontotemporal dementia modeling begins at C9orf72 (historically annotated 3110043O21Rik), the mouse ortholog of human C9ORF72, which encodes a DENN-domain guanine nucleotide exchange factor that partners stoichiometrically with SMCR8 and WDR41 to govern RAB GTPase cycling, ULK1-dependent autophagy initiation, and lysosomal trafficking. Disease arises from a GGGGCC hexanucleotide repeat expansion in intron 1 acting through three intertwined routes: transcriptional silencing and haploinsufficiency, sequestration of RNA-binding proteins into sense and antisense foci, and repeat-associated non-AUG translation into dipeptide repeat species including poly(GA), poly(GR), and poly(PR). Allele choice therefore encodes the hypothesis. Germline or conditional nulls interrogate loss of function, which in mice yields myeloid and microglial lysosomal defects plus autoimmunity rather than frank motor neuron death, repeat-expansion knockin or BAC alleles test gain of function, and humanized locus replacement enables antisense oligonucleotide and RNA-targeting campaigns. Match allele class to endpoint, pairing neuronal or myeloid Cre drivers with cell-type-restricted questions.
C9orf72 null animals are straightforward to genotype and phenotype when survival is acceptable. They remain a standard background for pharmacology, biomarker, and rescue studies.
What C9orf72 Knockout mouse models are available?
ingenious targeting laboratory offers 1 distinct C9orf72 knockout catalog mouse model. Researchers can order pre-developed catalog lines or request a custom mouse model, including humanized, knockin, and transgenic variations with verified germline transmission.
Catalog table
| Model | Type | Category | Availability | Catalog # | Action |
|---|---|---|---|---|---|
| C9orf72-KO | Knockout | KO/CKO mice, disease model mice | sperm cryopreservation | KO 201246 | Inquire |
Why this approach
A conventional knockout answers whether the gene is required broadly. When your target tissue is the organ of interest, a global null can still be informative if viability is acceptable and you want the simplest genotype. If the null is harsh, a floxed allele with a regional Cre is the safer long term platform.
Pricing and quotes
The C9orf72 Knockout lines listed above are catalog models. Send us the catalog number and our team confirms current availability, pricing, and whether the line ships cryopreserved or live.
If your study needs a C9orf72 allele configuration that is not listed above, our scientific team designs and generates it. Model generation quotes return in about twenty four hours with project milestones and pricing.
FAQ
What C9orf72 Knockout mouse models are available?
When C9orf72 Knockout lines are in catalog, we ship from inventory. If your configuration is not listed, we design the allele to order. Common paths include conditional knockout, constitutive knockout, humanized, knockin, and transgenic options, with documented germline transmission and United States QC.
Is C9orf72 knockout embryonic lethal in mice?
It depends on background and allele design. Some C9orf72 germline knockouts are viable. Others need conditional alleles or mixed backgrounds. We review publications and our own experience, then recommend floxed versus null before you commit.
Which Cre driver is best for experiments?
Driver choice depends on onset timing, recombination efficiency, and known leak. We map your organ and cell type to a short list of proven Cre lines, then talk through reporter crosses and controls. We favor drivers with strong community validation for your tissue.
Do you ship live C9orf72 animals?
When catalog lines are live, we ship with health certificates and QC documentation. If your exact combo is not listed, we quote a generation project with cryo or live dispatch depending on cohort timing and geography.
How do I request a quote for C9orf72?
Use the catalog inquire buttons or the request quote form. Include your allele goal, Cre plan if any, strain background, and cohort size. A PhD led team responds with pricing, milestones, and the fastest path to experimental animals.