C57BL/6JCya-Abcb4em1flox/Cya
Common Name:
Abcb4-flox
Product ID:
S-CKO-04262
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Abcb4-flox
Strain ID
CKOCMP-18670-Abcb4-B6J-VA
Gene Name
Product ID
S-CKO-04262
Gene Alias
Mdr2; Pgy-2; Pgy2; mdr-2
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
5
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Abcb4em1flox/Cya mice (Catalog S-CKO-04262) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000003717
NCBI RefSeq
NM_008830
Target Region
Exon 4
Size of Effective Region
~1.8 kb
Detailed Document
Overview of Gene Research
Abcb4, also known as multidrug resistance protein 3 (MDR3), is a member of the ATP-binding cassette transporter superfamily. It is localized at the canalicular membrane of hepatocytes and mediates the translocation of phosphatidylcholine (PC) into bile. This function is crucial for solubilizing cholesterol into mixed micelles and preventing bile acid toxicity towards hepatobiliary epithelia, thus protecting the hepatobiliary system from deleterious detergent and lithogenic properties of the bile [1,2,3,4,5].
In ABCB4 knockout mice, a model for low phospholipid-associated cholelithiasis (LPAC), the gallbladder bile has only trace amounts of phospholipids, is supersaturated with cholesterol, and shows “anhydrous” cholesterol crystallization. These mice exhibit features similar to human LPAC, such as a reduction or deficiency of phospholipids in bile, which helps to understand the pathogenesis of LPAC [6].
In conclusion, Abcb4 is essential for biliary phospholipid secretion. Studies using ABCB4 knockout mouse models have provided insights into the role of Abcb4 in diseases like LPAC, highlighting its significance in maintaining normal biliary function and preventing the development of certain liver and gallbladder diseases.
References：
1. Sticova, Eva, Jirsa, Milan. 2019. ABCB4 disease: Many faces of one gene deficiency. In Annals of hepatology, 19, 126-133. doi:10.1016/j.aohep.2019.09.010. https://pubmed.ncbi.nlm.nih.gov/31759867/
2. Prescher, Martin, Kroll, Tim, Schmitt, Lutz. . ABCB4/MDR3 in health and disease - at the crossroads of biochemistry and medicine. In Biological chemistry, 400, 1245-1259. doi:10.1515/hsz-2018-0441. https://pubmed.ncbi.nlm.nih.gov/30730833/
3. Reichert, Matthias Christian, Lammert, Frank. 2018. ABCB4 Gene Aberrations in Human Liver Disease: An Evolving Spectrum. In Seminars in liver disease, 38, 299-307. doi:10.1055/s-0038-1667299. https://pubmed.ncbi.nlm.nih.gov/30357767/
4. Stättermayer, Albert Friedrich, Halilbasic, Emina, Wrba, Fritz, Ferenci, Peter, Trauner, Michael. 2020. Variants in ABCB4 (MDR3) across the spectrum of cholestatic liver diseases in adults. In Journal of hepatology, 73, 651-663. doi:10.1016/j.jhep.2020.04.036. https://pubmed.ncbi.nlm.nih.gov/32376413/
5. Falguières, Thomas, Aït-Slimane, Tounsia, Housset, Chantal, Maurice, Michèle. 2014. ABCB4: Insights from pathobiology into therapy. In Clinics and research in hepatology and gastroenterology, 38, 557-63. doi:10.1016/j.clinre.2014.03.001. https://pubmed.ncbi.nlm.nih.gov/24953525/
6. Wang, Helen H, Portincasa, Piero, Liu, Min, Wang, David Q-H. 2022. Genetic Analysis of ABCB4 Mutations and Variants Related to the Pathogenesis and Pathophysiology of Low Phospholipid-Associated Cholelithiasis. In Genes, 13, . doi:10.3390/genes13061047. https://pubmed.ncbi.nlm.nih.gov/35741809/
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