Search results for ABCB4

Showing 13 results out of 18

×

Species

Types

Compartments

Reaction types

Search properties

Species

Types

Compartments

Reaction types

Search properties

Protein (5 results from a total of 10)

Identifier: R-HSA-400167
Species: Homo sapiens
Compartment: plasma membrane
Primary external reference: UniProt: ABCB4: P21439
Identifier: R-HSA-5678839
Species: Homo sapiens
Compartment: plasma membrane
Primary external reference: UniProt: P21439
Identifier: R-HSA-5678948
Species: Homo sapiens
Compartment: plasma membrane
Primary external reference: UniProt: P21439
Identifier: R-HSA-5678946
Species: Homo sapiens
Compartment: plasma membrane
Primary external reference: UniProt: P21439
Identifier: R-HSA-5678865
Species: Homo sapiens
Compartment: plasma membrane
Primary external reference: UniProt: P21439

DNA Sequence (1 results from a total of 1)

Identifier: R-HSA-5649955
Species: Homo sapiens
Compartment: nucleoplasm
Primary external reference: ENSEMBL: ENSG00000005471

Reaction (3 results from a total of 3)

Identifier: R-HSA-1989762
Species: Homo sapiens
Compartment: nucleoplasm, plasma membrane
The ABCB4 gene is transcribed to yield mRNA and the mRNA is translated to yield protein.
Identifier: R-HSA-5678706
Species: Homo sapiens
Compartment: extracellular region, plasma membrane
Multidrug resistance protein 3 (ATP-binding cassette sub-family B member 4, ABCB4 aka MDR3) mediates the ATP-dependent export of organic anions and drugs from hepatocytes into the canalicular lumen in the presence of bile salts. ABCB4 is especially important for the export of phospholipids such as phosphatidylcholine (PC) from the plasma membrane of hepatocytes (Morita et al. 2007). Biliary phospholipids associate with bile salts and cholesterol in mixed micelles, thereby reducing the detergent activity and cytotoxicity of bile salts and preventing cholesterol crystallisation. Thus, ABCB4 plays a crucial role in bile formation and lipid homeostasis (Morita & Terada 2014).
Identifier: R-HSA-5678749
Species: Homo sapiens
Compartment: cytosol, plasma membrane
Multidrug resistance protein 3 (ATP-binding cassette sub-family B member 4, ABCB4 aka MDR3) mediates the ATP-dependent export of organic anions, phospholipids and drugs from hepatocytes into the canalicular lumen in the presence of bile salts, especially the export of phospholipids such as phosphatidylcholine (PC). Biliary phospholipids associate with bile salts and cholesterol in mixed micelles, thereby reducing the detergent activity and cytotoxicity of bile salts and preventing cholesterol crystallisation. Thus, ABCB4 plays a crucial role in bile formation and lipid homeostasis.

Defects in ABCB4 result in a wide spectrum of cholestasis phenotypes, from progressive familial intrahepatic cholestasis 3 (PFIC3; MIM:602347), intrahepatic cholestasis of pregnancy 3 (ICP3; MIM:614972) to gallbladder disease 1 (GBD1; MIM:600803). Mutations causing PFIC3 include R957*, Y403H and V571Dfs*16 (de Vree et al. 1998, Degiorgio et al. 2007, Jacquemin et al. 1999). Mutations causing ICP3 include A546D, R144* and R590Q (Dixon et al. 2000, Bacq et al. 2009, Ziol et al. 2008). Mutations causing GBD1 include T175V, P1161S and R545G (Rosmorduc et al. 2001, Ziol et al. 2008).

Set (1 results from a total of 1)

Identifier: R-HSA-5678836
Species: Homo sapiens
Compartment: plasma membrane

Pathway (2 results from a total of 2)

Identifier: R-HSA-5678771
Species: Homo sapiens
Multidrug resistance protein 3 (ATP-binding cassette sub-family B member 4, ABCB4 aka MDR3) mediates the ATP-dependent export of organic anions, phospholipids and drugs from hepatocytes into the canalicular lumen in the presence of bile salts, especially the export of phospholipids such as phosphatidylcholine (PC). Biliary phospholipids associate with bile salts and cholesterol in mixed micelles, thereby reducing the detergent activity and cytotoxicity of bile salts and preventing cholesterol crystallisation. Thus, ABCB4 plays a crucial role in bile formation and lipid homeostasis. Defects in ABCB4 result in a wide spectrum of cholestasis phenotypes, from progressive familial intrahepatic cholestasis 3 (PFIC3; MIM:602347) and intrahepatic cholestasis of pregnancy 3 (ICP3; MIM:614972) to gallbladder disease 1 (GBD1; MIM:600803) (Jacquemin et al. 2001, Davit-Spraul et al. 2010, Jacquemin 2012). In PFIC3, the biliary phospholipid level is substantially decreased despite the presence of bile acids. Cholestasis may be caused by the toxicity of detergent bile salts that are not associated with phospholipids, leading to bile canaliculus and biliary epithelium damage. ICP3 is a reversible form of cholestasis in the third trimester of pregnancy and quickly disappears after childbearing. GBD1 is one of the major digestive diseases. Gallstones composed of cholesterol (cholelithiasis) are the common manifestations of GBD1 in western countries. Most people with gallstones remain asymptomatic throughout their lifetimes but approximately 10-50% of individuals eventually develop symptoms.
Identifier: R-HSA-1989781
Species: Homo sapiens
Compartment: cytosol, endoplasmic reticulum membrane, extracellular region, lipid droplet, mitochondrial inner membrane, mitochondrial matrix, mitochondrial outer membrane, nucleoplasm, peroxisomal matrix, peroxisomal membrane, plasma membrane
The set of genes regulated by PPAR-alpha is not fully known in humans, however many examples have been found in mice. Genes directly activated by PPAR-alpha contain peroxisome proliferator receptor elements (PPREs) in their promoters and include:
1) genes involved in fatty acid oxidation and ketogenesis (Acox1, Cyp4a, Acadm, Hmgcs2);
2) genes involved in fatty acid transport (Cd36, , Slc27a1, Fabp1, Cpt1a, Cpt2);
3) genes involved in producing fatty acids and very low density lipoproteins (Me1, Scd1);
4) genes encoding apolipoproteins (Apoa1, Apoa2, Apoa5);
5) genes involved in triglyceride clearance ( Angptl4);
6) genes involved in glycerol metabolism (Gpd1 in mouse);
7) genes involved in glucose metabolism (Pdk4);
8) genes involved in peroxisome proliferation (Pex11a);
9) genes involved in lipid storage (Plin, Adfp).
Many other genes are known to be regulated by PPAR-alpha but whether their regulation is direct or indirect remains to be found. These genes include: ACACA, FAS, SREBP1, FADS1, DGAT1, ABCA1, PLTP, ABCB4, UGT2B4, SULT2A1, Pnpla2, Acsl1, Slc27a4, many Acot genes, and others (reviewed in Rakhshandehroo et al. 2010).

Icon (1 results from a total of 1)

Species: Homo sapiens
Curator: Bijay Jassal
Designer: Cristoffer Sevilla
ABCB4 icon
Phosphatidylcholine translocator ABCB4
Cite Us!