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C57BL/6NCya-Lipaem1flox/Cya
Common Name:
Lipa-flox
Product ID:
S-CKO-17470
Background:
C57BL/6NCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Lipa-flox
Strain ID
CKOCMP-16889-Lipa-B6N-VA
Gene Name
Lipa
Product ID
S-CKO-17470
Gene Alias
Lal; Lip-1; Lip1
Background
C57BL/6NCya
NCBI ID
16889
Modification
Conditional knockout
Chromosome
19
Phenotype
MGI:96789
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/6NCya-Lipaem1flox/Cya mice (Catalog S-CKO-17470) 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
Click here to download >>
Overview of Gene Research
Lipa, also known as lipase A, encodes lysosomal acid lipase (LAL), which is crucial for the hydrolysis of both triglycerides (TGs) and cholesteryl esters (CEs) in the lysosome [3]. This enzyme is involved in lipid metabolism pathways, and its normal function is of great biological importance for maintaining lipid homeostasis. Genetic models, such as gene knockout models, can be used to study its function.

In Aspergillus flavus, knockout of the lipA gene led to suppressed growth, reduced formation of conidia and sclerotia, and down-regulation of aflatoxin B1 (AFB1), indicating its role in the development of A. flavus and AFB1 biosynthesis [1]. In non-alcoholic fatty liver disease (NAFLD) mouse models, exercise upregulated LIPA expression, increased its activity, and ameliorated lipid droplet metabolism disorder through the PLIN2-LIPA axis-mediated lipophagy [4]. Mutations in the LIPA gene in humans cause lysosomal acid lipase deficiency (LAL-D), with severe subtypes like Wolman disease. In Egyptian patients, novel LIPA variants were identified, expanding the spectrum of variants involved in the disease [3]. In cholesteryl ester storage disease (CESD) patients with LIPA gene mutations, there were significant changes in plasma levels and lipid composition of lipoproteins, increasing cardiovascular risk [2].

In conclusion, Lipa plays a vital role in lipid metabolism, affecting the growth and toxin production in fungi, lipid droplet metabolism in NAFLD, and lipoprotein composition in humans. The study of Lipa knockout or mutated models has enhanced our understanding of its functions in these biological processes and disease conditions, providing insights into potential therapeutic strategies for related diseases.

References:
1. Bai, Wenzhao, Feng, Tiejun, Lan, Faxiu, Liu, Yaju, Jia, Kunzhi. 2018. Study on the bio-function of lipA gene in Aspergillus flavus. In Genes & genomics, 41, 107-111. doi:10.1007/s13258-018-0744-7. https://pubmed.ncbi.nlm.nih.gov/30264213/
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
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