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C57BL/6JCya-Lmnaem1/Cya
Common Name:
Lmna-KO
Product ID:
S-KO-02895
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Lmna-KO
Strain ID
KOCMP-16905-Lmna-B6J-VA
Gene Name
Lmna
Product ID
S-KO-02895
Gene Alias
Dhe
Background
C57BL/6JCya
NCBI ID
16905
Modification
Conventional knockout
Chromosome
3
Phenotype
MGI:96794
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-Lmnaem1/Cya mice (Catalog S-KO-02895) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000029699
NCBI RefSeq
NM_001002011
Target Region
Exon 2~5
Size of Effective Region
~2.8 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Lmna, encoding lamin A/C, is a gene whose protein product is a component of the nuclear envelope. It regulates nuclear mechanostability and function, chromatin organization, and gene transcription [1]. These functions are crucial for normal cellular processes and the maintenance of tissue homeostasis. Mouse models, among other genetic models, have been invaluable in studying Lmna's role.

Mutations in Lmna cause a variety of diseases known as laminopathies, with LMNA-related cardiomyopathy being a common and highly penetrant inherited heart disease with a poor prognosis [1]. Mouse models have elucidated pathways linking Lmna dysfunction to fibrosis, arrhythmias, and myocardial remodeling, revealing that Lmna mutations disrupt nuclear envelope stability, activate the DNA damage response, and compromise chromatin organization and mechanotransduction [3]. Additionally, studies on Lmna-related cardiomyopathy show that it often presents early, with electrical abnormalities often preceding left ventricular dysfunction [2].

In conclusion, Lmna is essential for maintaining nuclear integrity and function, with its role extending to crucial cellular processes. Model-based research, especially using mouse models, has significantly contributed to understanding its role in LMNA-related cardiomyopathy. This knowledge is vital for advancing diagnosis, management strategies, and potentially developing targeted therapies for this and other laminopathy-related diseases [1,2,3].

References:

1. Wang, Yinuo, Dobreva, Gergana. 2023. Epigenetics in LMNA-Related Cardiomyopathy. In Cells, 12, . doi:10.3390/cells12050783. https://pubmed.ncbi.nlm.nih.gov/36899919/

2. Rosario, Karen Flores, Karra, Ravi, Amos, Kaitlyn, Kim, Han, Devore, Adam D. 2023. LMNA Cardiomyopathy: Important Considerations for the Heart Failure Clinician. In Journal of cardiac failure, 29, 1657-1666. doi:10.1016/j.cardfail.2023.08.016. https://pubmed.ncbi.nlm.nih.gov/37659618/

3. Rouhi, Leila. 2025. Cardiac phenotypes in LMNA mutations. In Current opinion in cardiology, 40, 131-138. doi:10.1097/HCO.0000000000001209. https://pubmed.ncbi.nlm.nih.gov/39998502/

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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