Coq8a Knockout mouse models
Coq8a, ortholog of human COQ8A (also known as ADCK3 and CABC1), encodes an atypical UbiB-family protein kinase-like ATPase that lacks canonical protein kinase activity but binds cardiolipin and coenzyme Q intermediates, uses ATP hydrolysis to support complex Q assembly and stability, and thereby acts as a regulator rather than a biosynthetic enzyme. Recessive human variants cause autosomal recessive cerebellar ataxia type 2 with Purkinje cell loss, exercise intolerance and variable seizures, and partial responsiveness to high-dose coenzyme Q10 supplementation is a defining clinical feature. Usefully for modeling, constitutive nulls are viable and develop cerebellar ataxia with Purkinje cell degeneration, so an unconditional allele is often sufficient, while Pcp2-Cre conditional deletion establishes cell autonomy and a patient missense knockin retains partial ATPase function for supplementation trials. The paralog Coq8b covers largely non-overlapping tissues, chiefly kidney, so it does not rescue cerebellar loss; confirm tissue-specific expression before invoking redundancy.
A conventional Coq8a knockout removes gene function in all cells that inherit the allele. It is a proven first pass for target validation when redundancy is low.
What Coq8a Knockout mouse models are available?
ingenious targeting laboratory offers 1 distinct Coq8a (ADCK3) 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 |
|---|---|---|---|---|---|
| Coq8a-KO | Knockout | KO/CKO mice | live | KO 200934 | 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 Coq8a 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 Coq8a 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 Coq8a Knockout mouse models are available?
When Coq8a 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 Coq8a knockout embryonic lethal in mice?
It depends on background and allele design. Some Coq8a 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 Coq8a 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 Coq8a?
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.