C57BL/6JCya-Abcb11em1flox/Cya
Common Name
Abcb11-flox
Product ID
S-CKO-10041
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-27413-Abcb11-B6J-VA
Status
When using this mouse strain in a publication, please cite “Abcb11-flox Mouse (Catalog S-CKO-10041) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
The standard delivery applies for a guaranteed minimum of three heterozygous carriers. Breeding services for homozygous carriers and/or specified sex are available.
Basic Information
Strain Name
Abcb11-flox
Strain ID
CKOCMP-27413-Abcb11-B6J-VA
Gene Name
Product ID
S-CKO-10041
Gene Alias
Bsep, PGY4, SPGP, ABC16, Lith1, PFIC2
Background
C57BL/6JCya
Gene Full Name
ATP-binding cassette, sub-family B (MDR/TAP), member 11
Modification
Conditional knockout
NCBI ID
Phenotype
Chromosome
Chr 2 (Mouse)
Application
--
Datasheet
Strain Description
Ensembl Transcript ID
ENSMUST00000102710
NCBI Transcript ID
NM_021022
Target Region
Exon 5
Size of Effective Region
~0.7 kb
Overview of Gene Research
Abcb11, also known as BSEP (bile salt export pump), is a member of the ATP-binding cassette transporter family. It is located on the apical membrane of hepatocytes and mediates the secretion of bile salts from hepatocytes into the bile. This process is the rate-limiting step of bile salt secretion and the main driving force of bile flow, thus driving and maintaining the enterohepatic circulation of bile salts [2,3].
Mutations in Abcb11 can lead to various liver diseases. In children, it may cause progressive familial intrahepatic cholestasis type 2 (PFIC2), presenting as severe jaundice and liver dysfunction. For example, a homozygous Abcb11 mutation c.386G>A (p.C129Y) in a patient with PFIC2 led to impaired trafficking of BSEP to the plasma membrane, reduced cell-surface expression, and decreased bile acid transport [5]. In adults, Abcb11 variants are associated with pregnancy-associated liver dysfunction and acute/episodic cholestasis. The common variant p.Val444Ala in Abcb11 confers an increased risk of drug-induced liver injury and intrahepatic cholestasis of pregnancy (ICP) [1,4].
In conclusion, Abcb11 is crucial for bile salt secretion and maintaining the enterohepatic circulation. Genetic studies, especially those on mutations in Abcb11, have revealed its significant role in the development of various liver diseases in both children and adults, providing insights into the molecular mechanisms underlying these diseases and potential directions for treatment.
References：
1. Nayagam, Jeremy S, Foskett, Pierre, Strautnieks, Sandra, Joshi, Deepak, Thompson, Richard J. 2022. Clinical phenotype of adult-onset liver disease in patients with variants in ABCB4, ABCB11, and ATP8B1. In Hepatology communications, 6, 2654-2664. doi:10.1002/hep4.2051. https://pubmed.ncbi.nlm.nih.gov/35894240/
2. Bull, Laura N, Thompson, Richard J. 2018. Progressive Familial Intrahepatic Cholestasis. In Clinics in liver disease, 22, 657-669. doi:10.1016/j.cld.2018.06.003. https://pubmed.ncbi.nlm.nih.gov/30266155/
3. Ren, Tengqi, Pang, Liwei, Dai, Wanlin, Wu, Shuodong, Kong, Jing. 2021. Regulatory mechanisms of the bile salt export pump (BSEP/ABCB11) and its role in related diseases. In Clinics and research in hepatology and gastroenterology, 45, 101641. doi:10.1016/j.clinre.2021.101641. https://pubmed.ncbi.nlm.nih.gov/33581308/
4. Nayagam, Jeremy S, Williamson, Catherine, Joshi, Deepak, Thompson, Richard J. 2020. Review article: liver disease in adults with variants in the cholestasis-related genes ABCB11, ABCB4 and ATP8B1. In Alimentary pharmacology & therapeutics, 52, 1628-1639. doi:10.1111/apt.16118. https://pubmed.ncbi.nlm.nih.gov/33070363/
5. Imagawa, Kazuo, Hayashi, Hisamitsu, Sabu, Yusuke, Kusuhara, Hiroyuki, Sumazaki, Ryo. 2018. Clinical phenotype and molecular analysis of a homozygous ABCB11 mutation responsible for progressive infantile cholestasis. In Journal of human genetics, 63, 569-577. doi:10.1038/s10038-018-0431-1. https://pubmed.ncbi.nlm.nih.gov/29507376/
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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