Conventional / global / constitutive KO
●1 Model Available
Coq9 Knockout mouse model
Browse 1 Coq9 mouse model including knockout variants from ingenious targeting laboratory. Contact us for availability and fast turnaround.
Why model Coq9
Coq9 functions as a lipid-binding partner rather than a catalyst, encoding the mouse ortholog of human COQ9, a protein with a START-like hydrophobic cavity that extracts coenzyme Q intermediates from the inner membrane bilayer and presents them to COQ7, forming a stable complex that is required for the C6-hydroxylation step and for overall stability of complex Q. Recessive human variants, including the well-studied R244X truncation modeled by the Coq9 R239X mouse, cause neonatal encephalomyopathy with demyelination, seizures and renal tubulopathy, accompanied by diagnostic accumulation of demethoxyubiquinone. The allele fork is clear: a knockin truncation reproduces the destabilized COQ7 interaction and progressive encephalopathy suitable for therapeutic testing with 2,4-dihydroxybenzoate, whereas a conditional null with Nes-Cre or renal drivers assigns tissue autonomy. No paralog substitutes for the lipid-binding function, so interpret residual coenzyme Q as dietary uptake, and quantify the demethoxyubiquinone to coenzyme Q ratio as the primary biochemical endpoint.
What Coq9 mouse models are available?
ingenious targeting laboratory offers 1 distinct Coq9 (COQ9) catalog mouse model, featuring conditional knockout and standard knockout. Researchers can order pre-developed catalog lines or request a custom mouse model, including humanized, knockin, and transgenic variations with verified germline transmission.
| Model Availability | Allele Class Options |
|---|---|
| 1 Ready Catalog Line | Conditional Knockout (cKO) |
| Custom Model Generation | Constitutive Knockout (KO) |
| Advanced Modifications | Humanized, Knockin, Transgenic |
- Quality Control
- Documented Germline Transmission
- Quality Control
- Strict United States QC Guidelines
- Quality Control
- Cryo or Live Dispatch Options
Coq9 model types
PathAvailability
Knockout
Conditional knockout
Knockin
Humanized
Transgenic / overexpression
Prefer the order form? Order. Need an allele that is not listed? Request a quote.
Coq9 allele types
Knockout, conditional, knockin, humanized, and related paths for Coq9 (human COQ9). Catalog lines ship when inventory exists. Everything else is a generation quote.
Knockout
Conditional KO (floxed / loxP)
Inducible conditional KO (CreER / tet)
Tissue specific KO
Specify tissue or Cre driver on quote
Knockout first (tm1a / IKMC)
Convertible floxed allele pathway
Double / compound KO
Multi allele / compound knockout project
Large scale / BAC deletion
BAC scale deletion or targeting
Knockin
Point mutation KI
cDNA KI
Reporter KI (GFP, YFP, RFP, mCherry, tdTomato, lacZ, luciferase)
Tag KI (FLAG, HA, Myc, V5)
Conditional KI (e.g. Rosa26 LSL)
LSL or conditional expression knockin
Gene replacement KI
Humanized
Full gene replacement
Partial / domain humanized
Domain or partial humanization scope
Immune checkpoint humanized
Checkpoint IO humanization when gene is a checkpoint target
Single humanized
Double / multi humanized
Multi humanized / combination IO project
Transgenic
Random integration transgenic
Safe harbor (ROSA26, HPRT, H11, Col1a1)
Overexpression transgenic
BAC transgenic
BAC transgenic or large fragment insert
Cre / recombinase
Constitutive Cre driver
Inducible Cre (CreER / tet)
Tamoxifen or dox inducible Cre
Dual recombinase (Cre+Dre / Cre+Flp)
Dual recombinase breeding scheme
Flp driver
Flp or FRT derivative allele pairing
Reporter
Reporter knockin (gene specific)
Standalone reporter catalog (lineage tracing, constitutive, dual reporter)
Catalog reporter lines not tied to a single gene allele
Species / background
Rat KO / KI / transgenic
Specify rat on quote
Rabbit models
Specify rabbit on quote
C57BL/6 (B6J / B6N)
Background substrain on quote
BALB/c
129, NOD, NSG, FVB, CD1, DBA/2
Background strain on quote
Available Coq9 catalog models
1 Coq9 line with QC documentation and technical support.
- Order
Coq9-KO
KnockoutKO/CKO mice
embryo cryopreservationKO 201259
Selected publications from ingenious targeting laboratory
Peer reviewed work involving Coq9 (COQ9) from ITL supported projects.
- Gene Therapy Corrects Mitochondrial Dysfunction in Hematopoietic Progenitor Cells and Fibroblasts from Coq9R239X Mice.Barriocanal-Casado E, Cueto-Urena C, Benabdellah K, Gutierrez-Guerrero A, Cobo M, Hidalgo-Gutierrez A, Rodriguez-Sevilla JJ, Martin F, Lopez LC. (2016). PLoS One 11: e0158344
- The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene.Luna-Sanchez M, Diaz-Casado E, Barca E, Tejada MA, Montilla-Garcia A, Cobos EJ, Escames G, Acuna-Castroviejo D, Quinzii CM, Lopez LC. (2015). EMBO Mol Med 7: 670-687
- Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency.Garcia-Corzo L, Luna-Sanchez M, Doerrier C, Garcia JA, Guaras A, Acin-Perez R, Bullejos-Peregrin J, Lopez A, Escames G, Enriquez JA, Acuña-Castroviejo D, López LC. (2013). Hum Mol Genet 22: 1233-1248
Looking for a Coq9 mouse model?
We have 1 Coq9 model available — including Knockout types. Contact us today for current availability and our fastest turnaround options. All models come with full QC documentation and technical support.
Best pricing in the industry. Get a quote in 24 hours. Our team of PhD scientists is available to help you select the right model for your research.
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About Coq9 Mouse Model Types
A Coq9 knockout mouse has the Coq9 gene permanently inactivated, enabling loss of function studies.
Modifications available
Jump to catalog backed pages for Coq9 organized by modification type. Each URL is indexable and matches common search patterns.