Logo
Homepage
Explore Our Models
My Cart
Contact
Subscribe
Models
Genetically Engineered Animals
Knockout Mice
Knockout Rats
Knockin Mice
Knockin Rats
Transgenic Mice
Transgenic Rats
Model Generation Techniques
Turboknockout<sup>®</sup> Gene Targeting
ES Cell Gene Targeting
Targeted Gene Editing
Regular Transgenic
PiggyBac Transgenesis
BAC Transgenic
Research Models
HUGO-GT™ Humanized Mice
Cre Mouse Lines
Humanized Target Gene Models
Metabolic Disease Models
Ophthalmic Disease Models
Neurological Disease Models
Autoimmune Disease Models
Immunodeficient Mouse Models
Humanized Immune System Mouse Models
Oncology & Immuno-oncology Models
Covid-19 Mouse Models
MouseAtlas Model Library
Knockout Cell Line Product Catalog
Tumor Cell Line Product Catalog
AAV Standard Product Catalog
Animal Supporting Services
Breeding Services
Cryopreservation & Recovery
Phenotyping Services
BAC Modification
Custom Cell Line Models
Induced Pluripotent Stem Cells (iPSCs)
Knockout Cell Lines
Knockin Cell Lines
Point Mutation Cell Lines
Overexpression Cell Lines
Virus Packaging
Adeno-associated Virus (AAV) Packaging
Lentivirus Packaging
Adenovirus Packaging
CRO Services
By Therapeutic Area
Oncology
Ophthalmology
Neuroscience
Metabolic & Cardiovascular Diseases
Autoimmune & Inflammatory
By Drug Type
AI-Powered AAV Discovery
Gene Therapy
Oligonucleotide Therapy
Antibody Therapy
Cell Immunotherapy
Resources
Promotion
Events & Webinars
Newsroom
Blogs & Insights
Resource Vault
Reference Databases
Peer-Reviewed Citations
Rare Disease Data Center
AbSeek
Cell iGeneEditor™ System
OriCell
Quality
Facility Overview
Animal Health & Welfare
Health Reports
About Us
Corporate Overview
Our Partners
Careers
Contact Us
Login
Request a Product Quote
Select products from our catalogs and submit your request. Our team will get back to you with detailed information.
Full Name
Email
Phone Number
Organization
Job Role
Country
Catalog Type
Product Name
Additional Comments
Cyagen values your privacy. We’d like to keep you informed about our latest offerings and insights. Your preferences:
You may unsubscribe from these communications at any time. See our Privacy Policy for details on opting out and data protection.
By clicking the button below, you consent to allow Cyagen to store and process the personal information submitted in this form to provide you the content requested.
C57BL/6JCya-Abcb11em1flox/Cya
Common Name:
Abcb11-flox
Product ID:
S-CKO-10041
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Abcb11-flox
Strain ID
CKOCMP-27413-Abcb11-B6J-VA
Gene Name
Abcb11
Product ID
S-CKO-10041
Gene Alias
ABC16; Bsep; Lith1; PFIC2; PGY4; SPGP
Background
C57BL/6JCya
NCBI ID
27413
Modification
Conditional knockout
Chromosome
2
Phenotype
MGI:1351619
Document
Click here to download >>
Application
--
More
Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Abcb11em1flox/Cya mice (Catalog S-CKO-10041) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000102710
NCBI RefSeq
NM_021022
Target Region
Exon 5
Size of Effective Region
~0.7 kb
Detailed Document
Click here to download >>
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
Model Library
Model Library
Resources
Resources
Animal Quality
Animal Quality
Get Support
Get Support
Address:
2255 Martin Avenue, Suite E Santa Clara, CA 95050-2709, US
Tel:
800-921-8930 (8-6pm PST)
+1408-963-0306 (lnt’l)
Fax:
408-969-0338
Email:
animal-service@cyagen.com
service@cyagen.us
CRO Services
OncologyOphthalmologyNeuroscienceMetabolic & CardiovascularAutoimmune & InflammatoryGene TherapyAntibody Therapy
About Us
Corporate OverviewOur PartnersCareersContact Us
Social Media
Disclaimer: Pricing and availability of our products and services vary by region. Listed prices are applicable to the specific countries. Please contact us for more information.
Copyright © 2025 Cyagen. All rights reserved.
Privacy Policy
Site Map
Stay Updated with the Latest from Cyagen
Get the latest news on our research models, CRO services, scientific resources, and special offers—tailored to your research needs and delivered straight to your inbox.
Full Name
Email
Organization
Country
Areas of Interest