C57BL/6JCya-Isca1em1flox/Cya
Common Name:
Isca1-flox
Product ID:
S-CKO-14391
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Isca1-flox
Strain ID
CKOCMP-69046-Isca1-B6J-VA
Gene Name
Product ID
S-CKO-14391
Gene Alias
1810010A06Rik; Hbld2
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
13
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Isca1em1flox/Cya mice (Catalog S-CKO-14391) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000057115
NCBI RefSeq
NM_026921
Target Region
Exon 3
Size of Effective Region
~1.1 kb
Detailed Document
Overview of Gene Research
Isca1, or Iron-sulfur cluster assembly 1, is an iron-sulfur (Fe/S) carrier protein. It plays a crucial role in the maturation of mitochondrial [4Fe-4S] proteins, accepting Fe/S from a scaffold protein and transferring it to target mitochondrial proteins. This process is essential for mitochondrial function, especially in energy metabolism pathways such as the mitochondrial respiratory chain [2,3,4].
In gene knockout studies, neuron-specific Isca1 knockout rats (Isca1flox/flox-NeuN-Cre) developed multiple mitochondrial dysfunction syndromes (MMDS5). These rats showed developmental retardation, epilepsy, memory impairment, massive neuronal death, mitochondrial damage, and reduced ATP production [1]. Myocardium-specific Isca1 knockout rats (Isca1flox/flox/α-MHC-Cre) suffered from cardiomyocyte mitochondria damage, iron metabolism disorder, myocardial oncosis, and eventually heart failure [5]. Knocking out Isca1 in rats led to abnormal embryo development at 8.5 days, early embryonic death, and affected the expression of key proteins in the mitochondrial respiratory chain complex [4].
In conclusion, Isca1 is essential for mitochondrial Fe4S4 biogenesis and normal energy metabolism. Gene knockout models in rats have revealed its significant role in preventing multiple mitochondrial dysfunction syndromes in the nervous system and heart. These models provide valuable insights into the pathogenesis of MMDS and other related mitochondrial diseases [1,4,5].
References：
1. Sheng, Hanxuan, Lu, Dan, Qi, Xiaolong, Zhang, Li, Zhang, Lianfeng. . A neuron-specific Isca1 knockout rat developments multiple mitochondrial dysfunction syndromes. In Animal models and experimental medicine, 6, 155-167. doi:10.1002/ame2.12318. https://pubmed.ncbi.nlm.nih.gov/37140997/
2. Suraci, Dafne, Saudino, Giovanni, Nasta, Veronica, Ciofi-Baffoni, Simone, Banci, Lucia. 2021. ISCA1 Orchestrates ISCA2 and NFU1 in the Maturation of Human Mitochondrial [4Fe-4S] Proteins. In Journal of molecular biology, 433, 166924. doi:10.1016/j.jmb.2021.166924. https://pubmed.ncbi.nlm.nih.gov/33711344/
3. Beilschmidt, Lena Kristina, Ollagnier de Choudens, Sandrine, Fournier, Marjorie, Puccio, Hélène, Martelli, Alain. 2017. ISCA1 is essential for mitochondrial Fe4S4 biogenesis in vivo. In Nature communications, 8, 15124. doi:10.1038/ncomms15124. https://pubmed.ncbi.nlm.nih.gov/28492233/
4. Yang, Xinlan, Lu, Dan, Zhang, Xu, Ma, Yuanwu, Zhang, Lianfeng. 2019. Knockout of ISCA1 causes early embryonic death in rats. In Animal models and experimental medicine, 2, 18-24. doi:10.1002/ame2.12059. https://pubmed.ncbi.nlm.nih.gov/31016283/
5. Ling, Yahao, Yang, Xinlan, Zhang, Xu, Lu, Dan, Zhang, Lianfeng. 2022. Myocardium-specific Isca1 knockout causes iron metabolism disorder and myocardial oncosis in rat. In Life sciences, 297, 120485. doi:10.1016/j.lfs.2022.120485. https://pubmed.ncbi.nlm.nih.gov/35304126/
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