C57BL/6JCya-Lipaem1flox/Cya
Common Name:
Lipa-flox
Product ID:
S-CKO-03401
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Lipa-flox
Strain ID
CKOCMP-16889-Lipa-B6J-VA
Gene Name
Product ID
S-CKO-03401
Gene Alias
Lal; Lip-1; Lip1
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
19
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Lipaem1flox/Cya mice (Catalog S-CKO-03401) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000049572
NCBI RefSeq
NM_021460
Target Region
Exon 3
Size of Effective Region
~0.6 kb
Detailed Document
Overview of Gene Research
LIPA, encoding lysosomal acid lipase (LAL), is crucial for the hydrolysis of triglycerides (TGs) and cholesteryl esters (CEs) in the lysosome [3]. This process is integral to lipid metabolism pathways, and the gene's proper function is of great biological importance in maintaining lipid homeostasis. Genetic models, such as gene knockout models, can be valuable in studying LIPA.
Mutations in the LIPA gene lead to LAL deficiency (LAL-D), with severe subtypes like Wolman disease (WD) [3]. In Egyptian patients, a novel missense variant in LIPA was identified, expanding the understanding of WD-related variants [3]. In cholesteryl ester storage disease (CESD) patients with LIPA gene mutations, there are significant impacts on plasma levels and lipid composition of lipoproteins, increasing cardiovascular risk [2]. Exercise can upregulate LIPA expression in a high-fat diet-induced non-alcoholic fatty liver disease (NAFLD) mouse model, suggesting a role for LIPA in lipid droplet metabolism disorder amelioration through the PLIN2-LIPA axis-mediated lipophagy [4]. Also, in Pseudomonas protegens Pf-5, ArgR directly inhibits lipA (a form of LIPA) transcription, revealing a regulatory mechanism [1].
In conclusion, LIPA is essential for lipid metabolism. Model-based research, including gene knockout models, has helped reveal its role in diseases such as WD, CESD, and NAFLD. Understanding LIPA's function provides insights into lipid-related disease mechanisms, potentially guiding the development of targeted therapies.
References:
1. Ying, Wei, Wang, Xing-Lian, Shi, Hong-Qiu, Yang, Jian-Yuan, Zha, Dai-Ming. 2019. ArgR directly inhibits lipA transcription in Pseudomonas protegens Pf-5. In Biochimie, 167, 34-41. doi:10.1016/j.biochi.2019.08.018. https://pubmed.ncbi.nlm.nih.gov/31491441/
2. Arnaboldi, Lorenzo, Ossoli, Alice, Giorgio, Eleonora, Calabresi, Laura, Gomaraschi, Monica. 2020. LIPA gene mutations affect the composition of lipoproteins: Enrichment in ACAT-derived cholesteryl esters. In Atherosclerosis, 297, 8-15. doi:10.1016/j.atherosclerosis.2020.01.026. https://pubmed.ncbi.nlm.nih.gov/32058863/
3. Elaraby, Nesma M, Galal, Eman Reda, Abdel-Hamid, Mohamed, Mounir, Samir M, Ashaat, Engy A. 2023. First LIPA Mutational Analysis in Egyptian Patients Reveals One Novel Variant: Wolman Disease. In Journal of molecular neuroscience : MN, 73, 598-607. doi:10.1007/s12031-023-02139-6. https://pubmed.ncbi.nlm.nih.gov/37470904/
4. Fang, Chunlu, Liu, Shujing, Yang, Wenqi, Peng, Shuang, Li, Liangming. 2024. Exercise ameliorates lipid droplet metabolism disorder by the PLIN2-LIPA axis-mediated lipophagy in mouse model of non-alcoholic fatty liver disease. In Biochimica et biophysica acta. Molecular basis of disease, 1870, 167045. doi:10.1016/j.bbadis.2024.167045. https://pubmed.ncbi.nlm.nih.gov/38306800/
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