- Catalog number
- PB9414
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- Bile Salt Export Pump (BSEP) is a protein which in humans is encoded by the ABCB 11 gene. It is a member of the superfamily of ATP-binding cassette (ABC) transporters, also known as ABCB11. It is mapped to chromosome 2q24. The BSEP protein is mainly expressed in the liver. ABCB11 is a gene associated with progressive familial intrahepatic cholestasis type 2 (PFIC2) which caused by mutations in the ABCB11 gene will increases the risk of hepatocellular carcinoma in early life.
- Synonyms
- Bile salt export pump; ATP-binding cassette sub-family B member 11; ABCB11; BSEP
- Gene Name
- ABCB11
- Gene ID
- 8647
- UniProt
- O95342
- Host
- Rabbit
- Reactivity
- Human, Mouse, Rat
- Cross Reactivity
- No cross-reactivity with other proteins
- Immunogen
- A synthetic peptide corresponding to a sequence at the C-terminus of human ABCB11, different from the related mouse and rat sequences by three amino acids.
- Clonality
- Polyclonal
- Tissue Specificity
- Expressed predominantly, if not exclusively in the liver, where it was further localized to the canalicular microvilli and to subcanalicular vesicles of the hepatocytes by in situ.
- Applications
- IF, IHC, WB
- Field of Research
- Cancer, Cancer Metabolism, Channels, Lipid and Lipoprotein Metabolism, Lipid Metabolism, Metabolic Signaling Pathway, Metabolic Signaling Pathways, Metabolism, Metabolism of Lipids and Lipoproteins, Pathways and Processes, Plasma Membrane, Signal Transduction
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- Involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes.
- References & Citations
- 1. Knisely AS, Strautnieks SS, Meier Y, Stieger B, Byrne JA, Portmann BC, Bull LN, Pawlikowska L, Bilezikçi B, Ozçay F, László A, Tiszlavicz L, Moore L, Raftos J, Arnell H, Fischler B, Németh A, Papadogiannakis N, Cielecka-Kuszyk J, Jankowska I, Pawłowska J, Melín-Aldana H, Emerick KM, Whitington PF, Mieli-Vergani G, Thompson RJ (August 2006) . Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 44 (2) : 478–86. 2. Noe J, Kullak-Ublick GA, Jochum W, Stieger B, Kerb R, Haberl M, Müllhaupt B, Meier PJ, Pauli-Magnus C (September 2005) . Impaired expression and function of the bile salt export pump due to three novel ABCB11 mutations in intrahepatic cholestasis. J. Hepatol. 43 (3) : 536–43. 3. Strautnieks SS, Bull LN, Knisely AS, Kocoshis SA, Dahl N, Arnell H, Sokal E, Dahan K, Childs S, Ling V, Tanner MS, Kagalwalla AF, Németh A, Pawlowska J, Baker A, Mieli-Vergani G, Freimer NB, Gardiner RM, Thompson RJ (November 1998) . A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis. Nat. Genet. 20 (3) : 233–8. 4. Strautnieks, S., Kagalwalla, A. F., Tanner, M. S., Knisely, A. S., Bull, L. N., Freimer, N. B., Kocoshis, S. A., Gardiner, R. M., Thompson, R. J.Identification of a second locus for progressive familial intrahepatic cholestasis on chromosome 2q24.Medizinische Genetik9: 16-only, 1997.
- Storage Conditions
- Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 146407 MW
- Observed Molecular Weight
- 146 kDa
- Specificity
- No cross reactivity with other proteins.
- Gene Name Synonym
- Bile salt export pump
- Subcellular Location
- Membrane; Multi-pass membrane protein.
- Sequence Similarities
- Belongs to the ABC transporter superfamily. ABCB family. Multidrug resistance exporter (TC 3.A.1.201) subfamily.
- Protein Name
- Bile salt export pump
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.