C57BL/6JCya-Dnajc19em1flox/Cya
Common Name
Dnajc19-flox
Product ID
S-CKO-13835
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-67713-Dnajc19-B6J-VA
When using this mouse strain in a publication, please cite “Dnajc19-flox Mouse (Catalog S-CKO-13835) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Basic Information
Strain Name
Dnajc19-flox
Strain ID
CKOCMP-67713-Dnajc19-B6J-VA
Gene Name
Product ID
S-CKO-13835
Gene Alias
1810055D05Rik, Tim14
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
Chr 3
Phenotype
Datasheet
Application
--
Strain Description
Ensembl Number
ENSMUST00000011029
NCBI RefSeq
NM_001026211
Target Region
Exon 2~5
Size of Effective Region
~2.0 kb
Overview of Gene Research
Dnajc19, a member of the DNAJ heat shock protein (Hsp40) family, is localized within the inner mitochondrial membrane. It plays a crucial role in regulating the function and localization of mitochondrial Hsp70 (MtHsp70) and is involved in mitochondrial protein import machinery [4,5]. It also forms a complex with prohibitins to regulate cardiolipin remodeling, which is essential for mitochondrial morphogenesis [3].
Mutations in DNAJC19 cause Dilated Cardiomyopathy with Ataxia Syndrome (DCMA). In human iPSC-derived cardiomyocytes with DNAJC19 mutations, there are altered mitochondrial structure, increased mitochondrial respiration, abnormal cristae formation, and mitochondrial dysfunction [2]. In NSCLC, high expression of DNAJC19 promotes cell growth and lung metastasis by regulating the PI3K/AKT signaling pathway, and knockdown of DNAJC19 inhibits these processes [1].
In conclusion, DNAJC19 is essential for mitochondrial function, morphogenesis, and biogenesis. Studies on DNAJC19, especially through gene-edited models like KO human embryonic stem cells [5], have provided insights into the pathogenesis of DCMA-related cardiomyopathy and revealed its potential as a therapeutic target in NSCLC.
References：
1. Zhou, Ji, Peng, Yang, Gao, Ying-Chun, Liu, Tao, Ren, Tao. 2021. Targeting DNAJC19 overcomes tumor growth and lung metastasis in NSCLC by regulating PI3K/AKT signaling. In Cancer cell international, 21, 338. doi:10.1186/s12935-021-02054-z. https://pubmed.ncbi.nlm.nih.gov/34217321/
2. Janz, Anna, Walz, Katharina, Cirnu, Alexandra, Duff, Henry J, Gerull, Brenda. 2023. Mutations in DNAJC19 cause altered mitochondrial structure and increased mitochondrial respiration in human iPSC-derived cardiomyocytes. In Molecular metabolism, 79, 101859. doi:10.1016/j.molmet.2023.101859. https://pubmed.ncbi.nlm.nih.gov/38142971/
3. Richter-Dennerlein, Ricarda, Korwitz, Anne, Haag, Mathias, Rugarli, Elena I, Langer, Thomas. 2014. DNAJC19, a mitochondrial cochaperone associated with cardiomyopathy, forms a complex with prohibitins to regulate cardiolipin remodeling. In Cell metabolism, 20, 158-71. doi:10.1016/j.cmet.2014.04.016. https://pubmed.ncbi.nlm.nih.gov/24856930/
4. Al Tuwaijri, Abeer, Alyafee, Yusra, Alharbi, Mashael, Umair, Muhammad, Alfadhel, Majid. 2022. Novel homozygous pathogenic mitochondrial DNAJC19 variant in a patient with dilated cardiomyopathy and global developmental delay. In Molecular genetics & genomic medicine, 10, e1969. doi:10.1002/mgg3.1969. https://pubmed.ncbi.nlm.nih.gov/35611801/
5. Chen, Guangduo, Bai, Rui, Huang, Pufeng, Ni, Jie, Chen, Lianglong. 2024. Generation of a homozygous DNAJC19 knockout human embryonic stem cell line by CRISPR/Cas9 system. In Stem cell research, 77, 103427. doi:10.1016/j.scr.2024.103427. https://pubmed.ncbi.nlm.nih.gov/38696852/
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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