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C57BL/6JCya-Uqcrc1em1flox/Cya
Common Name:
Uqcrc1-flox
Product ID:
S-CKO-18085
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Price:
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Basic Information
Strain Name
Uqcrc1-flox
Strain ID
CKOCMP-22273-Uqcrc1-B6J-VB
Gene Name
Uqcrc1
Product ID
S-CKO-18085
Gene Alias
1110032G10Rik
Background
C57BL/6JCya
NCBI ID
22273
Modification
Conditional knockout
Chromosome
9
Phenotype
MGI:107876
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-Uqcrc1em1flox/Cya mice (Catalog S-CKO-18085) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000026743
NCBI RefSeq
NM_025407
Target Region
Exon 3~6
Size of Effective Region
~3.9 kb
Detailed Document
Click here to download >>
Overview of Gene Research
UQCRC1, also known as ubiquinol-cytochrome c reductase core protein 1, is a nuclear-encoded protein located in the inner mitochondrial membrane and is an evolutionarily conserved core subunit of mitochondrial respiratory chain complex III [1,2,3,4,5]. It is crucial for mitochondrial function, which is essential for cellular metabolism, and is involved in processes like apoptosis regulation [1]. Genetic models such as Drosophila and mouse models are valuable for studying its function [1,2].

In Drosophila, neuronal knockdown of uqcrc1 leads to age-dependent parkinsonism-resembling defects, including dopaminergic neuron reduction and locomotor decline, which can be improved by UQCRC1 expression [1]. In mouse models, UQCRC1 p.Tyr314Ser knock-in mice exhibit age-dependent locomotor defects, dopaminergic neuronal loss, peripheral neuropathy, impaired respiratory chain complex III activity, and aberrant mitochondrial ultrastructures in nigral neurons. Also, levodopa injection can improve motor dysfunction in these mutant mice [2].

In conclusion, UQCRC1 is vital for mitochondrial function and plays a role in regulating apoptosis. Model-based research, especially using mouse models, has revealed its significance in Parkinson's-like phenotypes, expanding our understanding of the link between mitochondrial complex III dysfunction and Parkinson's disease [1,2].

References:
1. Hung, Yu-Chien, Huang, Kuan-Lin, Chen, Po-Lin, Lin, Chin-Hsien, Chan, Chih-Chiang. . UQCRC1 engages cytochrome c for neuronal apoptotic cell death. In Cell reports, 36, 109729. doi:10.1016/j.celrep.2021.109729. https://pubmed.ncbi.nlm.nih.gov/34551295/
2. Lin, Chin-Hsien, Tsai, Pei-I, Lin, Han-Yi, Farrer, Matthew, Wu, Ruey-Meei. . Mitochondrial UQCRC1 mutations cause autosomal dominant parkinsonism with polyneuropathy. In Brain : a journal of neurology, 143, 3352-3373. doi:10.1093/brain/awaa279. https://pubmed.ncbi.nlm.nih.gov/33141179/
3. Li, Wenhua, Wubulikasimu, Gulinaizaier, Zhao, Xiaoying, Zhu, Xiaodong, Chen, Zhiyu. 2019. UQCRC1 downregulation is correlated with lymph node metastasis and poor prognosis in CRC. In European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology, 45, 1005-1010. doi:10.1016/j.ejso.2019.02.025. https://pubmed.ncbi.nlm.nih.gov/30842031/
4. Wang, Qing, Li, Mengge, Gan, Yu, Zhang, Zhigang, Tu, Hong. 2020. Mitochondrial Protein UQCRC1 is Oncogenic and a Potential Therapeutic Target for Pancreatic Cancer. In Theranostics, 10, 2141-2157. doi:10.7150/thno.38704. https://pubmed.ncbi.nlm.nih.gov/32089737/
5. Fernandez-Mosquera, Lorena, Yambire, King Faisal, Couto, Renata, Milosevic, Ira, Raimundo, Nuno. 2019. Mitochondrial respiratory chain deficiency inhibits lysosomal hydrolysis. In Autophagy, 15, 1572-1591. doi:10.1080/15548627.2019.1586256. https://pubmed.ncbi.nlm.nih.gov/30917721/
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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