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C57BL/6JCya-Ercc8em1flox/Cya
Common Name:
Ercc8-flox
Product ID:
S-CKO-15302
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Ercc8-flox
Strain ID
CKOCMP-71991-Ercc8-B6J-VA
Gene Name
Ercc8
Product ID
S-CKO-15302
Gene Alias
2410022P04Rik; 2810431L23Rik; 4631412O06Rik; B130065P18Rik; Ckn1; Csa
Background
C57BL/6JCya
NCBI ID
71991
Modification
Conditional knockout
Chromosome
13
Phenotype
MGI:1919241
Document
Click here to download >>
Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Ercc8em1flox/Cya mice (Catalog S-CKO-15302) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000054835
NCBI RefSeq
NM_028042
Target Region
Exon 4
Size of Effective Region
~1.6 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Ercc8, also known as Cockayne syndrome type A (CSA), plays a critical role in the nucleotide excision repair (NER) complex [2,3,5]. The NER pathway is essential for repairing DNA damage, ensuring the restoration of basic DNA functions and cellular life activities. This makes Ercc8 biologically important in maintaining genomic stability. Genetic models, such as gene knockout (KO) or conditional knockout (CKO) mouse models, can be valuable for studying its functions.

In esophageal cancer, ERCC8 was identified as a novel cisplatin-resistant gene. It may contribute to cisplatin resistance through binding to damaged DNA for nucleotide excision repair. However, it has little effect on the proliferation and migration of esophageal cancer cells in vitro [1].

In a consanguineous Pakistani family, a novel homozygous missense mutation in ERCC8 co-segregated with cerebellar ataxia. The mutation affected protein structure, stability, and likely impaired complex formation [2]. A frameshift mutation in ERCC8 was associated with keratoconus and congenital cataracts, as it led to degradation of mutant proteins, reduced DNA damage repair ability, decreased cell viability, and abnormal cell responses [3].

Novel ERCC8 variants were found in Vietnamese siblings with Cockayne syndrome without UV-sensitivity, and a compound heterozygous mutation of ERCC8 was responsible for Cockayne syndrome in a Chinese family, expanding the genetic spectrum of the disease [4,5].

In gastric cancer, individual and joint expressions of ERCC6 and ERCC8 were associated with clinicopathological parameters and prognosis, and they were mainly involved in the NER pathway and regulation of the PI3K/AKT/mTOR pathway [6]. ERCC8 was also identified as one of the pleiotropic genes shared between amyotrophic lateral sclerosis and Parkinson's disease, enriched in the negative regulation of neuron projection development [7].

In conclusion, Ercc8 is crucial for DNA repair through its role in the NER pathway. Studies using various genetic models have revealed its implications in multiple diseases, including esophageal cancer, cerebellar ataxia, keratoconus with congenital cataracts, Cockayne syndrome, gastric cancer, and comorbidities between amyotrophic lateral sclerosis and Parkinson's disease. Understanding Ercc8's functions provides insights into disease mechanisms and potential therapeutic targets.

References:
1. Sui, Xue, Tang, Xiaolong, Wu, Xi, Liu, Yongshuo. 2022. Identification of ERCC8 as a novel cisplatin-resistant gene in esophageal cancer based on genome-scale Nuclease technology screening. In Biochemical and biophysical research communications, 593, 84-92. doi:10.1016/j.bbrc.2022.01.033. https://pubmed.ncbi.nlm.nih.gov/35063774/
2. Gauhar, Zeeshan, Tejwani, Leon, Abdullah, Uzma, Lim, Janghoo, Raja, Ghazala K. 2022. A Novel Missense Mutation in ERCC8 Co-Segregates with Cerebellar Ataxia in a Consanguineous Pakistani Family. In Cells, 11, . doi:10.3390/cells11193090. https://pubmed.ncbi.nlm.nih.gov/36231052/
3. Hao, Xiao-Dan, Yao, Yi-Zhi, Xu, Kai-Ge, Xu, Wen-Hua, Zhang, Jing-Jing. . Insufficient Dose of ERCC8 Protein Caused by a Frameshift Mutation Is Associated With Keratoconus With Congenital Cataracts. In Investigative ophthalmology & visual science, 63, 1. doi:10.1167/iovs.63.13.1. https://pubmed.ncbi.nlm.nih.gov/36454558/
4. Duong, Nguyen Thuy, Dinh, Tran Huu, Möhl, Britta S, Matsumoto, Naomichi, Meinke, Peter. 2022. Cockayne syndrome without UV-sensitivity in Vietnamese siblings with novel ERCC8 variants. In Aging, 14, 5299-5310. doi:10.18632/aging.204139. https://pubmed.ncbi.nlm.nih.gov/35748794/
5. Liu, Meng-Wei, Hu, Cheng-Feng, Jin, Jie-Yuan, Li, Ya-Li, Zhu, Lei. 2024. A compound heterozygous mutation of ERCC8 is responsible for a family with Cockayne syndrome. In Molecular biology reports, 51, 371. doi:10.1007/s11033-024-09235-9. https://pubmed.ncbi.nlm.nih.gov/38411728/
6. Chen, Jing, Li, Liang, Sun, Liping, Yuan, Yuan, Jing, Jingjing. 2021. Associations of individual and joint expressions of ERCC6 and ERCC8 with clinicopathological parameters and prognosis of gastric cancer. In PeerJ, 9, e11791. doi:10.7717/peerj.11791. https://pubmed.ncbi.nlm.nih.gov/34316408/
7. Tian, Ye, Ma, Guochen, Li, Haoqi, Xiong, Jingyuan, Cheng, Guo. 2023. Shared Genetics and Comorbid Genes of Amyotrophic Lateral Sclerosis and Parkinson's Disease. In Movement disorders : official journal of the Movement Disorder Society, 38, 1813-1821. doi:10.1002/mds.29572. https://pubmed.ncbi.nlm.nih.gov/37534731/
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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