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ABCB11 Membrane Protein Introduction

Introduction of ABCB11

ABCB11 encoded by ABCB11 gene, also known as BSEP, is a member of the superfamily of ATP-binding cassette (ABC) transporters which transport various molecules across extra- and intracellular membranes. There are seven subfamilies in ABC superfamily named as ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White, respectively. BSEP possesses 12 transmembrane domains and belongs to MDR/TAP subfamily. It is most expressed in the canalicular membrane of hepatocytes.

Basic Information of ABCB11
Protein Name Bile salt export pump
Gene Name ABCB11
Aliases BSEP, PGY4, SPGP, ABC16, BRIC2, PFIC2, PFIC-2
Organism Homo sapiens (Human)
UniProt ID O95342
Transmembrane Times 12
Length (aa) 1321
Sequence MSDSVILRSIKKFGEENDGFESDKSYNNDKKSRLQDEKKGDGVRVGFFQLFRFSSSTDIWLMFVGSLCAFLHGIAQPGVLLIFGTMTDVFIDYDVELQELQIPGKACVNNTIVWTNSSLNQNMTNGTRCGLLNIESEMIKFASYYAGIAVAVLITGYIQICFWVIAAARQIQKMRKFYFRRIMRMEIGWFDCNSVGELNTRFSDDINKINDAIADQMALFIQRMTSTICGFLLGFFRGWKLTLVIISVSPLIGIGAATIGLSVSKFTDYELKAYAKAGVVADEVISSMRTVAAFGGEKREVERYEKNLVFAQRWGIRKGIVMGFFTGFVWCLIFLCYALAFWYGSTLVLDEGEYTPGTLVQIFLSVIVGALNLGNASPCLEAFATGRAAATSIFETIDRKPIIDCMSEDGYKLDRIKGEIEFHNVTFHYPSRPEVKILNDLNMVIKPGEMTALVGPSGAGKSTALQLIQRFYDPCEGMVTVDGHDIRSLNIQWLRDQIGIVEQEPVLFSTTIAENIRYGREDATMEDIVQAAKEANAYNFIMDLPQQFDTLVGEGGGQMSGGQKQRVAIARALIRNPKILLLDMATSALDNESEAMVQEVLSKIQHGHTIISVAHRLSTVRAADTIIGFEHGTAVERGTHEELLERKGVYFTLVTLQSQGNQALNEEDIKDATEDDMLARTFSRGSYQDSLRASIRQRSKSQLSYLVHEPPLAVVDHKSTYEEDRKDKDIPVQEEVEPAPVRRILKFSAPEWPYMLVGSVGAAVNGTVTPLYAFLFSQILGTFSIPDKEEQRSQINGVCLLFVAMGCVSLFTQFLQGYAFAKSGELLTKRLRKFGFRAMLGQDIAWFDDLRNSPGALTTRLATDASQVQGAAGSQIGMIVNSFTNVTVAMIIAFSFSWKLSLVILCFFPFLALSGATQTRMLTGFASRDKQALEMVGQITNEALSNIRTVAGIGKERRFIEALETELEKPFKTAIQKANIYGFCFAFAQCIMFIANSASYRYGGYLISNEGLHFSYVFRVISAVVLSATALGRAFSYTPSYAKAKISAARFFQLLDRQPPISVYNTAGEKWDNFQGKIDFVDCKFTYPSRPDSQVLNGLSVSISPGQTLAFVGSSGCGKSTSIQLLERFYDPDQGKVMIDGHDSKKVNVQFLRSNIGIVSQEPVLFACSIMDNIKYGDNTKEIPMERVIAAAKQAQLHDFVMSLPEKYETNVGSQGSQLSRGEKQRIAIARAIVRDPKILLLDEATSALDTESEKTVQVALDKAREGRTCIVIAHRLSTIQNADIIAVMAQGVVIEKGTHEELMAQKGAYYKLVTTGSPIS

Function of ABCB11 Membrane Protein

ABCB11 is an ATP-dependent transporter of conjugated bile acids such as taurine-amidated and acyl-glucuronides. It is also reported as a high-affinity bile salt transporter such as glycochenodeoxycholate, glycocholate and taurochenodeoxycholate. ABCB11 is a major regulator of bile formation and bile flow, which plays an essential role in maintaining normal liver functions. The lacking ABCB11 function can decrease biliary bile salt secretion and bile flow, as well as the accumulation of the bile salts in hepatocytes, resulting in hepatotoxicity or cholestasis in humans. It has been revealed that mutations of ABCB11 have an association with the progression of cholestatic liver disease such as progressive familial intrahepatic cholestasis type 2 (PFIC2). And PFIC2 may increase the susceptibility of hepatocellular carcinoma in early life. Moreover, the variants in ABCB11 have been also revealed a role in the progression of anti-tuberculosis drug-induced cholestatic liver injury. Besides, ABCB11 also affect drug absorption by regulating the production of bile salt. It can bind to biliary cholesterol and phospholipids forming the micelles which promote drugs solubilization and absorption by the intestine.

Hypothetical mechanism of canalicular lipid excretion. Fig.1 Hypothetical mechanism of canalicular lipid excretion. (Elferink, 2007)

Application of ABCB11 Membrane Protein in Literature

  1. Sharma A., et al. Spectrum of genomic variations in Indian patients with progressive familial intrahepatic cholestasis. BMC Gastroenterol. 2018, 18(1): 107. PubMed ID: 29973134

    The study shows nine major genomic variations in ATP8B1, ABCB11 and ABCB4 genes associated with one-third of Indian children with progressive familial intrahepatic cholestasis (PFIC).

  2. Imagawa K., et al. Clinical phenotype and molecular analysis of a homozygous ABCB11 mutation responsible for progressive infantile cholestasis. Journal of Human Genetics. 2018, 63(5): 569-577. PubMed ID: 29507376

    The study indicates that the variant c.386G>A (p.C129Y) in ABCB11 is associated with progression of familial intrahepatic cholestasis type 2 with an impaired biliary excretion from hepatocytes and the inexistence of canalicular BSEP expression in liver histological assessments.

  3. Besheer T., et al. Diagnosis of cirrhosis in patients with chronic hepatitis C genotype 4: Role of ABCB11 genotype polymorphism and plasma bile acid levels. Turkish Journal of Gastroenterology. 2018, 29(3): 299-307. PubMed ID: 29755014

    The study reveals that the Egyptian patients with chronic hepatitis C genotype 4 who carry CC genotype of ABCB11 SNP 1331T > C and present high plasma bile acid levels are linked to advanced hepatic fibrosis.

  4. Wang N.L., et al. Splicing analysis of rare/novel synonymous or intronic variants identified in ABCB11 heterozygotes presenting as progressive intrahepatic cholestasis with low γ-glutamyltransferase. Hepatology Research. 2018, 48(7): 574-584. PubMed ID: 29316097

    The study identifies five rare synonymous or intronic variants in ABCB11 heterozygotes, which are involved in the progression of intrahepatic cholestasis with low γ-glutamyltransferase.

  5. Dixon P.H., et al. An expanded role for heterozygous mutations of ABCB4, ABCB11, ATP8B1, ABCC2 and TJP2 in intrahepatic cholestasis of pregnancy. Sci Rep. 2017, 7(1): 11823. PubMed ID: 28924228

    The study expands known variants in ABCB4 and ABCB11 and identifies the roles in intrahepatic cholestasis of pregnancy (ICP) for mutations in ATP8B1 and ABCC2.

ABCB11 Preparation Options

To obtain the soluble and functional target protein, the versatile Magic™ membrane protein production platform in Creative Biolabs enables many flexible options, from which you can always find a better match for your particular project. Aided by our versatile Magic™ anti-membrane protein antibody discovery platform, we also provide customized anti-ABCB11 antibody development services.


As a forward-looking research institute as well as a leading custom service provider in the field of membrane protein, Creative Biolabs has won good reputation among our worldwide customers for successfully accomplishing numerous challenging projects including generation of many functional membrane proteins. Please feel free to contact us for more information.

Reference

  1. Elferink R P and Paulusma C C. (2007). Function and pathophysiological importance of ABCB4 (MDR3 P-glycoprotein). Pflügers Archiv: European Journal of Physiology. 453(5): 601-610.

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