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C57BL/6JCya-Coq8bem1flox/Cya
Common Name:
Coq8b-flox
Product ID:
S-CKO-16586
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Coq8b-flox
Strain ID
CKOCMP-76889-Coq8b-B6J-VA
Gene Name
Coq8b
Product ID
S-CKO-16586
Gene Alias
0610012P18Rik; Adck4
Background
C57BL/6JCya
NCBI ID
76889
Modification
Conditional knockout
Chromosome
7
Phenotype
MGI:1924139
Document
Click here to download >>
Application
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More
Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Coq8bem1flox/Cya mice (Catalog S-CKO-16586) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000003860
NCBI RefSeq
NM_133770
Target Region
Exon 6~7
Size of Effective Region
~1.6 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Coq8b, also previously termed ADCK4, is a gene involved in the biosynthesis of coenzyme Q10 (CoQ10) [1-9]. CoQ10 is a key component of oxidative phosphorylation in the mitochondria, playing a vital role in energy production and maintaining mitochondrial function [1,4].

Mutations in Coq8b are mainly associated with steroid-resistant nephrotic syndrome (SRNS) and glomerulopathies. In Chinese children, it is one of the common causes of adolescent-onset proteinuria and/or chronic kidney disease (CKD) of unknown etiology [2]. Patients with Coq8b mutations often present with proteinuria and/or advanced CKD at diagnosis, and renal biopsy usually shows focal segmental glomerulosclerosis [2]. Early detection of Coq8b nephropathy, followed by CoQ10 supplementation combined with an angiotensin-converting enzyme (ACE) inhibitor, could slow the progression of renal dysfunction [2]. Kidney transplantation in patients with Coq8b-related end-stage renal disease (ESRD) has a low recurrence risk of proteinuria, but graft rejection should be monitored [3]. Additionally, bi-allelic variants in Coq8b may also lead to non-syndromic retinitis pigmentosa, possibly by impairing CoQ10 biosynthesis [4], and a common polymorphism in Coq8b may act as a modifier of thoracic aortic aneurysm severity [5].

In conclusion, Coq8b is essential for CoQ10 biosynthesis and mitochondrial function. Research on Coq8b-related mutations has significant implications for understanding and managing diseases such as SRNS-related glomerulopathies, non-syndromic retinitis pigmentosa, and thoracic aortic aneurysm [1-9].

References:
1. Drovandi, Stefania, Lipska-Ziętkiewicz, Beata S, Ozaltin, Fatih, Ariceta, Gema, Schaefer, Franz. 2022. Variation of the clinical spectrum and genotype-phenotype associations in Coenzyme Q10 deficiency associated glomerulopathy. In Kidney international, 102, 592-603. doi:10.1016/j.kint.2022.02.040. https://pubmed.ncbi.nlm.nih.gov/35483523/
2. Song, Xiaoxiang, Fang, Xiaoyan, Tang, Xiaoshan, Xu, Hong, Rao, Jia. 2020. COQ8B nephropathy: Early detection and optimal treatment. In Molecular genetics & genomic medicine, 8, e1360. doi:10.1002/mgg3.1360. https://pubmed.ncbi.nlm.nih.gov/32543055/
3. Zeng, Shuhan, Xu, Yuanyuan, Cheng, Cheng, Chen, Lizhi, Jiang, Xiaoyun. 2022. COQ8B glomerular nephropathy: Outcomes after kidney transplantation and analysis of characteristics in Chinese population. In Frontiers in pediatrics, 10, 938863. doi:10.3389/fped.2022.938863. https://pubmed.ncbi.nlm.nih.gov/36034551/
4. Iglesias-Romero, Ana Belén, Kaminska, Karolina, Quinodoz, Mathieu, Santos, Cristina, Rivolta, Carlo. 2024. Bi-allelic variants in COQ8B, a gene involved in the biosynthesis of coenzyme Q10, lead to non-syndromic retinitis pigmentosa. In American journal of human genetics, 111, 2299-2306. doi:10.1016/j.ajhg.2024.08.005. https://pubmed.ncbi.nlm.nih.gov/39226897/
5. Landis, Benjamin J, Lai, Dongbing, Guo, Dong-Chuan, Hinton, Robert B, Ware, Stephanie M. 2021. Identification of a common polymorphism in COQ8B acting as a modifier of thoracic aortic aneurysm severity. In HGG advances, 3, . doi:10.1016/j.xhgg.2021.100057. https://pubmed.ncbi.nlm.nih.gov/34917985/
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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