C57BL/6NCya-Kcnv2em1/Cya
Common Name
Kcnv2-KO
Product ID
S-KO-07205
Backgroud
C57BL/6NCya
Strain ID
KOCMP-240595-Kcnv2-B6N-VA
Status
When using this mouse strain in a publication, please cite “Kcnv2-KO Mouse (Catalog S-KO-07205) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
The standard delivery applies for a guaranteed minimum of three heterozygous carriers. Breeding services for homozygous carriers and/or specified sex are available.
Basic Information
Strain Name
Kcnv2-KO
Strain ID
KOCMP-240595-Kcnv2-B6N-VA
Gene Name
Product ID
S-KO-07205
Gene Alias
KV11.1
Background
C57BL/6NCya
Gene Full Name
potassium channel, subfamily V, member 2
Modification
Conventional knockout
NCBI ID
Phenotype
Chromosome
Chr 19 (Mouse)
Application
--
Datasheet
Strain Description
Ensembl Transcript ID
ENSMUST00000056708
NCBI Transcript ID
NM_183179
Target Region
Exon 1~2
Size of Effective Region
~14.6 kb
Overview of Gene Research
Kcnv2 encodes Kv8.2, a voltage-gated potassium channel subunit. It acts as a modulator by shifting the activation range of the K+ channels in photoreceptor inner segments, playing a crucial role in the normal function of the retina [1].
Mutations in Kcnv2 are associated with an autosomal recessive cone-rod dystrophy known as KCNV2-associated retinopathy or “cone dystrophy with supernormal rod responses”. Patients with this condition typically show decreased visual acuity, reduced color vision, photophobia, nyctalopia, and nystagmus. The disease progresses slowly, and legal blindness may be reached before the age of 25. Full-field electroretinograms (ERGs) show pathognomonic waveform features, such as a disproportionate increase in b-wave amplitude with a relatively small light intensity increase, delayed a-wave and b-wave peak times, and a higher than normal b:a wave ratio. Retinal imaging reveals features like bull's eye maculopathy pattern in fundus autofluorescence (FAF) and variable foveal disruption on optical coherence tomography (OCT) [1,2,3,4].
In conclusion, Kcnv2 is essential for the normal function of retinal photoreceptor inner segments. The study of KCNV2-associated retinopathy, often through clinical and genetic analysis of patients rather than traditional KO/CKO mouse models in the provided references, has provided insights into the disease mechanism. Understanding Kcnv2's role in this disease may help develop future therapeutic strategies for this currently untreatable condition [1].
References:
1. Guimaraes, Thales A C De, Georgiou, Michalis, Robson, Anthony G, Michaelides, Michel. 2020. KCNV2 retinopathy: clinical features, molecular genetics and directions for future therapy. In Ophthalmic genetics, 41, 208-215. doi:10.1080/13816810.2020.1766087. https://pubmed.ncbi.nlm.nih.gov/32441199/
2. Georgiou, Michalis, Robson, Anthony G, Fujinami, Kaoru, Héon, Elise, Michaelides, Michel. 2020. KCNV2-Associated Retinopathy: Genetics, Electrophysiology, and Clinical Course-KCNV2 Study Group Report 1. In American journal of ophthalmology, 225, 95-107. doi:10.1016/j.ajo.2020.11.022. https://pubmed.ncbi.nlm.nih.gov/33309813/
3. Sakti, Dhimas H, Cornish, Elisa E, Ali, Haipha, Jamieson, Robyn V, Grigg, John R. 2024. Natural history and biomarkers of KCNV2-associated retinopathy. In Clinical & experimental ophthalmology, 52, 528-544. doi:10.1111/ceo.14373. https://pubmed.ncbi.nlm.nih.gov/38443311/
4. Georgiou, Michalis, Fujinami, Kaoru, Vincent, Ajoy, Zrenner, Eberhart, Michaelides, Michel. 2021. KCNV2-Associated Retinopathy: Detailed Retinal Phenotype and Structural Endpoints-KCNV2 Study Group Report 2. In American journal of ophthalmology, 230, 1-11. doi:10.1016/j.ajo.2021.03.004. https://pubmed.ncbi.nlm.nih.gov/33737031/
Quality Control Standard
Sperm Test
Pre-cryopreservation: Measurement of sperm concentration, determination of sperm viability.
Post-cryopreservation: A vial of cryopreserved sperms is selected for in-vitro fertilization from each batch.
Environmental Standards:SPF
Available Region:Global
Source:Cyagen
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